Coronary syndromes, arrhythmias, valvular disease, heart failure, congenital defects, and vascular conditions - core content for MRCP, PLAB, and UKMLA.
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Hypertension
Sustained elevation of systemic arterial blood pressure (clinic BP ≥140/90 mmHg or ABPM/HBPM ≥135/85 mmHg). Affects ~30% of UK adults and is the leading modifiable risk factor for cardiovascular disease.
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Key Facts
NICE CG136 defines hypertension as clinic BP ≥140/90 mmHg AND ABPM/HBPM daytime average ≥135/85 mmHg
Stage 1: clinic BP 140/90–159/99 mmHg, ABPM 135/85–149/94 mmHg
Stage 2: clinic BP 160/100–179/119 mmHg, ABPM 150/95 mmHg or higher
Stage 3 (severe): clinic systolic BP ≥180 mmHg or diastolic BP ≥120 mmHg
First-line treatment: ACEi/ARB if <55 years or diabetic; CCB if ≥55 years or Black African/Caribbean
QRISK3 should be used for cardiovascular risk assessment in all hypertensive patients
Secondary causes account for ~5-10% of cases: renal artery stenosis, phaeochromocytoma, Conn syndrome, Cushing syndrome
Overview
Key Facts
Hypertension is the most common modifiable cardiovascular risk factor, affecting approximately 30% of adults in the UK. It is defined by NICE (CG136/NG136) as a sustained clinic blood pressure ≥140/90 mmHg confirmed by ambulatory (ABPM) or home blood pressure monitoring (HBPM) with a daytime average ≥135/85 mmHg.
Epidemiology
Prevalence: ~30% of UK adults (~14.4 million people)
Only ~60% of hypertensives are diagnosed; of those, ~40% achieve target BP
More common in men <65 years; women overtake after menopause
Higher prevalence in Black African/Caribbean populations
Accounts for ~50% of coronary heart disease and ~60% of strokes
Aetiology
Primary (essential) hypertension (~90-95%):
Multifactorial: genetic predisposition, obesity, high salt intake, excessive alcohol, physical inactivity, stress
Activation of the renin-angiotensin-aldosterone system (RAAS)
Sympathetic nervous system overactivity
Endothelial dysfunction with reduced nitric oxide bioavailability
Sodium retention and volume expansion
Arterial stiffness and vascular remodelling
Target organ damage affects heart (LVH, heart failure), brain (stroke), kidneys (nephrosclerosis), and eyes (hypertensive retinopathy)
Clinical Presentation
Typical Presentation
Hypertension is usually asymptomatic and detected incidentally during routine health checks. Symptoms typically only occur with severe hypertension or end-organ damage.
Blood pressure remaining above target despite optimal doses of three antihypertensive agents including a diuretic. Affects ~10-15% of treated hypertensives.
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Key Facts
Definition: BP above target despite ≥3 antihypertensives at optimal doses including a diuretic
Prevalence: affects ~10-15% of treated hypertensive patients in the UK
NICE step 4: add spironolactone 25-50mg OD if potassium ≤4.5 mmol/L
PATHWAY-2 trial: spironolactone was superior to doxazosin, bisoprolol, and placebo for resistant hypertension
Exclude pseudo-resistance: white coat effect, non-adherence, inadequate doses, incorrect technique
Secondary causes must be investigated: renal artery stenosis, primary hyperaldosteronism, phaeochromocytoma
Medication adherence can be assessed with directly observed therapy or urine/serum drug screening
Renal denervation remains investigational but SPYRAL HTN-ON MED trial showed promising results
Overview
Key Facts
Resistant hypertension is defined as blood pressure that remains above target (≥140/90 mmHg clinic or ≥135/85 mmHg ABPM) despite the use of optimal or maximally tolerated doses of three antihypertensive drugs from different classes, one of which should be a diuretic.
Epidemiology
Affects approximately 10-15% of treated hypertensive patients
More common in older patients, those with obesity, diabetes, CKD, and Black African/Caribbean ethnicity
Associated with significantly higher cardiovascular risk compared to controlled hypertension
Aetiology
Pseudo-resistance (most common):
Poor medication adherence (~50% of apparent resistant cases)
White coat effect
Suboptimal drug doses
Incorrect BP measurement technique
True resistance:
Volume overload (excess salt intake, inadequate diuretic therapy)
Severe hypertension (usually SBP >180 and/or DBP >120 mmHg) with acute end-organ damage requiring immediate but controlled BP reduction.
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Key Facts
Definition: severe hypertension with evidence of acute end-organ damage (brain, heart, kidneys, eyes, aorta)
Differs from hypertensive urgency: urgency = severe BP WITHOUT end-organ damage
Target: reduce MAP by no more than 25% in first hour, then to 160/100 mmHg over 2-6 hours
First-line IV agents: labetalol, sodium nitroprusside, GTN infusion, nicardipine
Malignant hypertension: severe hypertension with grade III/IV retinopathy (haemorrhages, papilloedema)
Avoid rapid BP reduction: risk of watershed cerebral infarction, coronary or renal hypoperfusion
Common presentations: hypertensive encephalopathy, aortic dissection, acute pulmonary oedema, eclampsia
Mortality: untreated malignant hypertension has ~90% 1-year mortality
Overview
Key Facts
A hypertensive emergency is severe hypertension (typically SBP >180 mmHg and/or DBP >120 mmHg) accompanied by evidence of acute, ongoing end-organ damage. This requires immediate but controlled blood pressure reduction, typically in an ICU/HDU setting.
Epidemiology
Affects ~1-2% of hypertensive patients
More common in men, Black African/Caribbean ethnicity, and those with poor medication adherence
UK incidence: approximately 1-5 per 100,000 per year
Declining incidence due to improved hypertension management
Aetiology
Most commonly occurs in patients with poorly controlled essential hypertension
Non-adherence to antihypertensive medications is the commonest precipitant
Predictable chest pain/discomfort caused by myocardial ischaemia during exertion or stress, relieved by rest or GTN. Due to fixed atherosclerotic coronary artery stenosis.
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Key Facts
NICE CG126/NG106: recommends CT coronary angiography as first-line investigation for stable chest pain
First-line symptom relief: sublingual GTN spray + either beta-blocker (bisoprolol 2.5-10mg OD) or CCB (amlodipine 5-10mg OD)
Secondary prevention: aspirin 75mg OD + atorvastatin 80mg OD + ACEi (if diabetes, HTN, CKD, or LV dysfunction)
GTN relieves symptoms within 1-3 minutes; if pain persists >15 minutes, suspect ACS
ISCHEMIA trial: invasive strategy did not reduce death/MI compared to optimal medical therapy in stable CAD
Revascularisation indicated for refractory symptoms despite optimal medical therapy, or left main stem disease
CCS classification grades angina severity I-IV based on functional limitation
Prevalence: ~3-4% of UK adults; more common in men and increases with age
Overview
Key Facts
Stable angina is characterised by predictable, exertion-related chest discomfort caused by fixed coronary artery atherosclerotic stenosis causing myocardial ischaemia. It is relieved by rest or sublingual GTN.
Epidemiology
Prevalence: ~2 million people in the UK
More common in men; female incidence rises post-menopause
Incidence increases with age: ~5-7% in those aged 65-74
Spectrum of acute myocardial ischaemia encompassing unstable angina, NSTEMI, and STEMI. Caused by atherosclerotic plaque rupture with thrombus formation.
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Acute coronary syndrome (ACS) is an umbrella term for conditions caused by sudden reduction in coronary blood flow due to atherosclerotic plaque disruption with superimposed thrombus formation. It encompasses unstable angina (UA), non-ST elevation myocardial infarction (NSTEMI), and ST elevation myocardial infarction (STEMI).
Epidemiology
~100,000 hospital admissions per year in England for ACS
Leading cause of death worldwide; ~70,000 deaths per year in the UK from coronary heart disease
Male:female ratio ~2:1; women present ~10 years later than men
In-hospital mortality: STEMI ~7%, NSTEMI ~5%, UA ~2%
Aetiology
Atherosclerotic plaque rupture/erosion with superimposed thrombus formation (>90%)
Less common: coronary artery spasm, coronary dissection (spontaneous or iatrogenic), coronary embolism, vasculitis, cocaine use, supply-demand mismatch (type 2 MI)
ST-elevation myocardial infarction caused by complete thrombotic occlusion of a coronary artery, resulting in transmural myocardial necrosis requiring emergency reperfusion.
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Key Facts
ECG criteria: ST elevation ≥2mm in ≥2 contiguous chest leads or ≥1mm in limb leads, or new LBBB
Primary PCI is the gold standard: door-to-balloon time <90 minutes, first-medical-contact-to-device <120 minutes
Thrombolysis if PPCI unavailable within 120 min: tenecteplase IV bolus (weight-adjusted)
Rescue PCI: if thrombolysis fails (persistent ST elevation and ongoing pain at 60-90 min post-lysis)
Anterior STEMI (LAD occlusion): worst prognosis, highest risk of LV dysfunction and heart failure
Complications: arrhythmia (VF most common cause of death in first 48h), cardiogenic shock, free wall rupture, VSD, papillary muscle rupture
30-day mortality: ~8-10% overall; <5% with timely PPCI
Overview
Key Facts
STEMI is the most severe manifestation of acute coronary syndrome, caused by complete occlusion of a coronary artery by thrombus superimposed on a ruptured or eroded atherosclerotic plaque. This leads to transmural myocardial infarction requiring emergency reperfusion therapy.
Epidemiology
~30,000 STEMI admissions per year in England
Male:female ratio ~3:1
Peak incidence: men 55-64, women 65-74
In-hospital mortality: ~7% (improved from >20% in pre-PCI era)
UK has established PPCI networks providing 24/7 access in most regions
Aetiology
Atherosclerotic plaque rupture/erosion with complete thrombotic occlusion (>95%)
Non-ST elevation myocardial infarction characterised by troponin elevation without persistent ST elevation, caused by subtotal coronary occlusion or distal embolisation.
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Key Facts
Diagnosis: elevated high-sensitivity troponin with ischaemic symptoms BUT without persistent ST elevation on ECG
ECG changes: ST depression, T-wave inversion, or may be normal; NEVER persistent ST elevation
Risk stratification: use GRACE score to guide timing of invasive management
NICE NG185: angiography within 72 hours for intermediate/high risk; within 24 hours if very high risk
Antiplatelet: aspirin 300mg + ticagrelor 180mg loading (PLATO trial - superior to clopidogrel)
Anticoagulation: fondaparinux 2.5mg SC OD preferred (OASIS-5 trial - lower bleeding vs enoxaparin)
More common than STEMI and carries significant 6-month mortality (~12%)
NSTEMI is a form of acute coronary syndrome characterised by myocardial necrosis (elevated troponin) in the setting of ischaemic symptoms, without persistent ST elevation on ECG. It is typically caused by subtotal coronary occlusion or distal microembolisation.
Epidemiology
NSTEMI is now more common than STEMI, accounting for ~60% of all acute MI
~70,000 NSTEMI admissions per year in England
Incidence increasing, partly due to improved hs-troponin sensitivity
Clinical syndrome where the heart is unable to pump sufficiently to meet the body's metabolic demands, or can only do so at elevated filling pressures. Classified by ejection fraction.
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Key Facts
Prevalence: ~900,000 people in the UK; lifetime risk ~20%
PARADIGM-HF: sacubitril/valsartan superior to enalapril in reducing CV death and HF hospitalisation
DAPA-HF/EMPEROR-Reduced: SGLT2 inhibitors reduce HF hospitalisation and CV death regardless of diabetes status
Device therapy: CRT if LBBB + QRS ≥150ms + EF ≤35%; ICD if EF ≤35% despite OMT
NICE NG106: updated guidelines recommend early initiation of all four drug classes
Overview
Key Facts
Heart failure is a complex clinical syndrome resulting from any structural or functional cardiac disorder that impairs the ability of the ventricle to fill with or eject blood. It is classified by left ventricular ejection fraction (LVEF) into HFrEF, HFmrEF, and HFpEF.
Epidemiology
Prevalence: ~1-2% of UK adults; ~900,000 people affected
Incidence: ~200,000 new diagnoses per year in the UK
Increases with age: ~10% in those >70 years
5-year mortality: ~50% (worse than many cancers)
Leading cause of hospitalisation in those >65 years
Rapid onset or worsening of heart failure symptoms requiring urgent medical intervention. May present as de novo acute HF or acute decompensation of chronic HF.
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Before discharge: ensure optimisation of HF medications; arrange follow-up within 2 weeks
Overview
Key Facts
Acute heart failure (AHF) is the rapid onset or worsening of signs and symptoms of heart failure. It may present as a first episode (de novo) or more commonly as an acute decompensation of chronic heart failure (ADCHF). It is a medical emergency requiring urgent assessment and treatment.
Epidemiology
~80,000 admissions per year in England
Accounts for ~5% of all emergency medical admissions
Mean age at presentation: ~75 years
In-hospital mortality: ~5-10%
30-day readmission rate: ~25%
1-year mortality post-discharge: ~25-30%
Aetiology
Precipitants of acute decompensation:
Acute coronary syndrome
Arrhythmia (especially new-onset AF with rapid ventricular rate)
Medication non-adherence or dietary indiscretion
Infection/sepsis
Uncontrolled hypertension
Acute valvular dysfunction (MR, AR)
Pulmonary embolism
Renal failure
Anaemia
Thyrotoxicosis
Pathophysiology
Acute increase in LV filling pressures
pulmonary congestion
pulmonary oedema
Neurohormonal activation (RAAS, SNS) causes vasoconstriction and fluid retention
Heart failure with LVEF ≥50%, characterised by diastolic dysfunction with impaired ventricular relaxation and filling. Accounts for ~50% of all heart failure cases.
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Key Facts
Definition: signs/symptoms of HF with LVEF ≥50% and evidence of diastolic dysfunction or raised filling pressures
Epidemiology: accounts for ~50% of all HF; more common in elderly women with hypertension, obesity, AF, and diabetes
Diagnosis: elevated NT-proBNP + echocardiographic evidence of diastolic dysfunction (E/e' ratio, LA enlargement)
H2FPEF and HFA-PEFF scores aid diagnosis in uncertain cases
SGLT2 inhibitors: empagliflozin (EMPEROR-Preserved) and dapagliflozin (DELIVER) - first drugs to show benefit
No other drug class has shown mortality benefit in HFpEF (unlike HFrEF)
Management focuses on: diuretics for congestion, treating comorbidities (HTN, AF, diabetes, obesity), SGLT2i
Prognosis: similar or slightly better than HFrEF; 5-year mortality ~40-50%
Overview
Key Facts
Heart failure with preserved ejection fraction (HFpEF) is a clinical syndrome characterised by signs and symptoms of heart failure, preserved LVEF (≥50%), and evidence of structural heart disease and/or diastolic dysfunction. It was previously termed 'diastolic heart failure.'
Epidemiology
Accounts for ~50% of all heart failure cases (proportion increasing)
Prevalence increases significantly with age: >10% in those >80 years
Female predominance (~60% women)
Strongly associated with hypertension, obesity, diabetes, and atrial fibrillation
Commonest sustained cardiac arrhythmia, characterised by rapid, irregular atrial activation replacing normal sinus rhythm. Major risk factor for stroke requiring anticoagulation assessment.
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Key Facts
Prevalence: ~1.4 million people in the UK; increases with age (~10% in those >75 years)
ECG: irregularly irregular rhythm, absent P waves, variable ventricular rate
Stroke risk: 5-fold increased; assess with CHA₂DS₂-VASc score - anticoagulate if score ≥2 (men) or ≥3 (women)
NICE NG196: DOACs preferred over warfarin for stroke prevention (apixaban, rivaroxaban, edoxaban, dabigatran)
Rate control first-line (NICE): beta-blocker (bisoprolol) or rate-limiting CCB (diltiazem); target resting HR <110 bpm
Rhythm control: consider if symptomatic despite rate control; flecainide (if no structural heart disease) or amiodarone
AF ablation: pulmonary vein isolation; consider for paroxysmal or persistent AF refractory to/intolerant of drugs (CASTLE-AF, CABANA)
Bleeding risk: assess with ORBIT score (NOT HAS-BLED per NICE) - should not prevent anticoagulation if indicated
Overview
Key Facts
Atrial fibrillation (AF) is the most common sustained cardiac arrhythmia, characterised by disorganised atrial electrical activity leading to irregular ventricular response. It is a major cause of stroke and heart failure.
Epidemiology
~1.4 million people in the UK (~2% of general population)
Prevalence: <1% in <60 years; ~10% in >75 years
Lifetime risk: ~25% for those >40 years
AF-related strokes are more severe with higher mortality than non-AF strokes
Dose reduction: apixaban 2.5mg BD if ≥2 of: age ≥80, weight ≤60kg, Cr ≥133
Warfarin if DOAC contraindicated or mechanical heart valve (target INR 2-3)
Do NOT use aspirin alone for stroke prevention in AF
Left atrial appendage occlusion (Watchman device): if anticoagulation contraindicated
2. Rate control (first-line per NICE):
Beta-blocker: bisoprolol 2.5-10mg OD (first-line)
Rate-limiting CCB: diltiazem 120-360mg MR OD (not with beta-blocker if HFrEF)
Digoxin: add-on if monotherapy insufficient; or first-line if sedentary/HF
Target resting HR <110 bpm (RACE-II trial: lenient rate control non-inferior to strict)
3. Rhythm control:
Consider if symptomatic despite adequate rate control, or as first-line if young/paroxysmal/reversible cause
DC cardioversion: if AF <48h or adequately anticoagulated ≥3 weeks (or TOE excludes thrombus)
Pharmacological cardioversion: flecainide 300mg PO (pill-in-pocket) or IV (if no structural heart disease); amiodarone IV if structural heart disease or HF
Macro-re-entrant atrial tachycardia with regular atrial rate ~300 bpm, typically with 2:1 AV block giving ventricular rate ~150 bpm. Characterised by sawtooth flutter waves on ECG.
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Key Facts
ECG: sawtooth flutter waves (F waves) best seen in leads II, III, aVF and V1; atrial rate ~300 bpm
Typical flutter: counter-clockwise re-entry circuit through the cavotricuspid isthmus (CTI) in right atrium
2:1 AV block most common: ventricular rate ~150 bpm; if rate exactly 150, consider flutter
Stroke risk: similar to AF - anticoagulate using CHA₂DS₂-VASc score (same thresholds as AF)
Rate control: same approach as AF (beta-blocker, diltiazem, digoxin)
Electrical cardioversion: effective; low energy often sufficient (50-100J)
Often coexists with AF: ~30-50% of flutter patients develop AF over time
Overview
Key Facts
Atrial flutter is a macro-re-entrant atrial tachycardia characterised by an organised atrial rhythm, typically at a rate of ~300 bpm, with characteristic sawtooth flutter waves on ECG. The ventricular rate depends on the degree of AV conduction (most commonly 2:1, giving ~150 bpm).
Epidemiology
Less common than AF; incidence ~0.08% per year
Male:female ratio ~2:1
Increases with age; peak incidence >80 years
Often coexists with or precedes AF (~30-50% develop AF)
Shares similar risk factors with AF
Aetiology
Similar to AF: hypertension, IHD, heart failure, valvular disease, COPD, post-cardiac surgery
Often occurs post-AF ablation or with class IC antiarrhythmic drugs (flecainide can convert AF to flutter)
Pathophysiology
Typical (CTI-dependent) flutter: counter-clockwise macro-re-entrant circuit in the right atrium using the cavotricuspid isthmus as a critical isthmus
Atypical flutter: non-CTI-dependent circuits; may involve left atrium or right atrial scars (post-surgical)
Regular atrial activation at ~300 bpm → AV node provides conduction block (2:1, 3:1, 4:1, or variable)
1:1 conduction is rare but dangerous (ventricular rate 300 bpm)
Clinical Presentation
Typical Presentation
Palpitations (regular, rapid)
Dyspnoea
Fatigue
Dizziness or presyncope
Chest discomfort
May present with heart failure or stroke (similar to AF)
Signs
Regular tachycardia (if fixed AV block ratio)
If variable block: irregularly irregular (can mimic AF)
Rapid, regular jugular venous 'flutter' waves
Signs of haemodynamic compromise if 1:1 conduction
Umbrella term for tachyarrhythmias originating above the ventricles, most commonly AVNRT and AVRT. Presents as regular narrow-complex tachycardia at 140-250 bpm.
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Key Facts
Most common types: AVNRT (~60%) and AVRT (~30%, including WPW); also includes atrial tachycardia
Pill-in-pocket: flecainide 100-300mg or verapamil 80-120mg for infrequent episodes
Avoid AV nodal blockers in pre-excited AF (wide-complex irregular tachycardia in WPW)
Overview
Key Facts
Supraventricular tachycardia (SVT) is a broad term for tachyarrhythmias involving tissue at or above the bundle of His. In clinical practice, it most commonly refers to paroxysmal SVT (PSVT), particularly AVNRT and AVRT.
Epidemiology
Prevalence: ~2.3 per 1,000 population
More common in women (2:1 for AVNRT)
AVNRT peak onset: 20-40 years
AVRT (WPW): often presents in younger patients
Rarely life-threatening unless associated with WPW and pre-excited AF
Aetiology
AVNRT: dual AV nodal pathways (slow and fast) forming a re-entry circuit within/around the AV node
AVRT: accessory pathway between atria and ventricles (overt = WPW with delta wave; concealed = no delta wave at rest)
Atrial tachycardia: ectopic focus or micro-re-entry in atrial tissue
Electrophysiology study: definitive diagnosis and ablation
Management
Non-pharmacological
Acute termination:
Modified Valsalva manoeuvre (REVERT trial): blow into 10mL syringe for 15 seconds in semi-recumbent position, then lie flat with legs elevated 45° for 15 seconds
Carotid sinus massage: with continuous ECG monitoring (avoid if carotid bruit/stenosis)
Facial ice immersion: (diving reflex)
Pharmacological
Acute management (haemodynamically stable):
Adenosine IV
6mg rapid push
12mg
12mg (flush with 20mL saline, via large-bore cannula, antecubital fossa)
Warn patient: transient chest tightness, flushing, sense of impending doom
Contraindicated in: asthma, 2nd/3rd degree heart block, severe hypotension
Caution with dipyridamole (potentiates effect) and carbamazepine
If adenosine contraindicated: verapamil 2.5-5mg IV over 2 minutes (avoid in HFrEF or concurrent beta-blocker)
Haemodynamically unstable:
Synchronised DC cardioversion: 70-120J biphasic
Prophylaxis (recurrent SVT):
Pill-in-pocket: flecainide 100-300mg PO (if no structural heart disease) + beta-blocker
Wide-complex tachycardia (QRS ≥120ms, rate >100 bpm) originating from the ventricles. Potentially life-threatening, may degenerate into VF. Most commonly occurs in structural heart disease.
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Key Facts
Definition: ≥3 consecutive ventricular beats at rate >100 bpm; non-sustained (<30s) vs sustained (≥30s or haemodynamic compromise)
ECG: broad-complex tachycardia (QRS ≥120ms); AV dissociation, capture/fusion beats are diagnostic
Key rule: assume any broad-complex tachycardia is VT until proven otherwise (especially if structural heart disease)
Most common cause: ischaemic heart disease with myocardial scar (re-entry mechanism)
Haemodynamically unstable: immediate synchronised DC cardioversion (120-150J biphasic)
Stable VT: IV amiodarone 300mg over 20-60 minutes, then 900mg over 24 hours
ICD implantation: for secondary prevention after sustained VT with structural heart disease, or primary prevention if EF ≤35%
Brugada criteria/Vereckei algorithm: help distinguish VT from SVT with aberrancy
Overview
Key Facts
Ventricular tachycardia (VT) is a wide-complex tachycardia originating from the ventricular myocardium or Purkinje system. It is a potentially life-threatening arrhythmia that may degenerate into ventricular fibrillation and cardiac arrest.
Epidemiology
Most commonly occurs in the context of structural heart disease
IHD with prior MI is the most common substrate
Non-sustained VT: common in DCM, HCM, and post-MI patients
Annual incidence of sudden cardiac death in the UK: ~100,000 out-of-hospital cardiac arrests
Aetiology
Ischaemic heart disease: myocardial scar from prior MI (most common)
Cardiomyopathies: DCM, HCM, ARVC
Inherited channelopathies: long QT syndrome, Brugada, CPVT
Chaotic, disorganised ventricular electrical activity producing no effective cardiac output. A shockable cardiac arrest rhythm requiring immediate defibrillation.
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Key Facts
ECG: chaotic, irregular waveform with no identifiable QRS complexes, varying amplitude and frequency
Clinical: cardiac arrest - no pulse, no cardiac output, loss of consciousness
Treatment: immediate defibrillation (unsynchronised shock 150-200J biphasic) - single most important intervention
Every minute delay in defibrillation reduces survival by ~7-10%
Commonest initial arrest rhythm in out-of-hospital cardiac arrest (~25%); most common cause of SCD
ALS algorithm
CPR
shock
CPR 2 min
shock; amiodarone 300mg IV after 3rd shock
Most common cause: acute myocardial ischaemia/infarction
Survival to discharge: ~8-10% for out-of-hospital VF arrest; better with bystander CPR + early defibrillation
Overview
Key Facts
Ventricular fibrillation (VF) is a chaotic cardiac rhythm originating from multiple re-entrant circuits within the ventricles, resulting in no coordinated ventricular contraction and therefore no cardiac output. It is invariably fatal without immediate defibrillation.
Epidemiology
~60,000 out-of-hospital cardiac arrests (OHCA) per year in the UK
VF/pulseless VT is the initial rhythm in ~25% of OHCA (declining proportion)
Overall OHCA survival to discharge: ~8-10%
VF/pVT has the best prognosis of all arrest rhythms if defibrillated promptly
Bystander CPR doubles survival; immediate defibrillation can achieve >50% survival
Aetiology
Acute myocardial ischaemia/infarction: most common cause
Polymorphic ventricular tachycardia occurring in the context of QT prolongation, characterised by twisting of the QRS axis around the isoelectric baseline. A life-threatening arrhythmia requiring urgent treatment.
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Key Facts
Polymorphic VT with a characteristic 'twisting of the points' pattern on ECG in the context of a prolonged QT interval
Common causes of QT prolongation: drugs (amiodarone, sotalol, erythromycin, antipsychotics, methadone), hypokalaemia, hypomagnesaemia, hypocalcaemia
First-line treatment: IV magnesium sulphate 2g (8 mmol) over 10-15 minutes regardless of serum Mg²⁺ level
Isoprenaline infusion or temporary overdrive pacing to increase heart rate and shorten QT interval if refractory
Avoid class Ia/III antiarrhythmics (amiodarone, procainamide, sotalol) as they prolong QT further
DC cardioversion if haemodynamically unstable or pulseless
Corrected QT (QTc) >500 ms carries significant risk of TdP
Congenital long QT syndromes: Romano-Ward (autosomal dominant), Jervell and Lange-Nielsen (autosomal recessive with deafness)
Overview
Key Facts
Torsades de Pointes (TdP) is a specific form of polymorphic ventricular tachycardia that occurs in the setting of QT interval prolongation. The name ('twisting of the points') describes the characteristic ECG pattern where the QRS complexes appear to rotate around the isoelectric baseline.
Epidemiology
Exact incidence is difficult to determine; many episodes are self-terminating
Drug-induced QT prolongation is the most common cause in clinical practice
Women have a longer baseline QTc and are at higher risk of drug-induced TdP
Congenital long QT syndrome prevalence: ~1 in 2,000
Prolonged PR interval (>200 ms) on ECG with all atrial impulses conducted to the ventricles. Usually benign and often an incidental finding requiring no treatment.
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Key Facts
Definition: PR interval >200 ms (>5 small squares) with every P wave followed by a QRS complex
Usually benign: common in athletes, vagal tone, and elderly patients
No treatment required in most cases unless symptomatic or progressing to higher-degree block
Marked first-degree block (PR >300 ms) may cause symptoms similar to pacemaker syndrome due to loss of AV synchrony
Prevalence: ~1-5% in young adults, increasing with age to ~5-10% in elderly
Overview
Key Facts
First degree heart block (first degree AV block) is characterised by delayed conduction through the AV node, resulting in a prolonged PR interval (>200 ms) on the ECG. All atrial impulses are conducted to the ventricles; no beats are 'dropped'.
Epidemiology
Prevalence: 1-5% in young adults, higher in athletes (~8%)
Increases with age: 5-10% in over-60s
More common in men
Found in ~2% of apparently healthy adults on routine ECG
Aetiology
Physiological: increased vagal tone (athletes, sleep)
Rhythm strip: confirm constant PR interval with 1:1 conduction
Bloods
U&Es: potassium (hyperkalaemia may impair conduction)
Digoxin level: if on digoxin
TFTs: hypothyroidism may contribute
Lyme serology: if epidemiological risk
Troponin: if acute presentation with concern for MI
Imaging
Echocardiography: if structural heart disease suspected
Special Tests
Holter monitor (24-48 hours): if intermittent symptoms or to assess for progression to higher-degree block
Exercise ECG: first-degree block that resolves with exercise is likely nodal and benign; persistent or worsening block during exercise suggests infra-nodal disease
Management
Non-pharmacological
Reassurance in asymptomatic patients with isolated first-degree block and narrow QRS
Review and adjust medications: reduce or stop AV nodal blocking drugs if clinically appropriate
Serial ECGs/monitoring: if concern about progression
Pharmacological
No specific pharmacological treatment for isolated first-degree block
Treat underlying cause (e.g., antibiotics for Lyme carditis: doxycycline 100mg BD for 21 days, or IV ceftriaxone 2g OD if severe)
Surgical/Interventional
Permanent pacemaker: rarely indicated; consider only if:
Marked PR prolongation (>300 ms) with symptoms (pseudo-pacemaker syndrome)
First-degree block with haemodynamic compromise demonstrated on echocardiography
Concomitant neuromuscular disease with progressive conduction abnormality
Referral Criteria
Symptomatic first-degree block: refer to cardiology
Evidence of progressive conduction disease: cardiology assessment
New first-degree block post-MI: monitored setting
Prognosis
Isolated first-degree block with narrow QRS: excellent prognosis, no increase in mortality
In the Framingham study, isolated first-degree block was associated with a modestly increased risk of AF and pacemaker implantation over long-term follow-up
First-degree block with wide QRS (infra-nodal): higher risk of progression to complete heart block
Post-inferior MI: usually transient, resolves within days
Lyme carditis: usually resolves completely with antibiotic treatment
Intermittent failure of conduction of atrial impulses to the ventricles, classified as Mobitz type I (Wenckebach) with progressive PR prolongation or Mobitz type II with fixed PR and dropped beats.
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Key Facts
Mobitz type I (Wenckebach): progressive PR prolongation until a beat is dropped; usually benign, block at AV node level
Mobitz type II: constant PR interval with sudden dropped QRS complexes; high risk of progressing to complete heart block
2:1 block: every other P wave conducted; cannot classify as type I or II from surface ECG alone
Mobitz II is an indication for permanent pacemaker implantation even in asymptomatic patients
Mobitz I in inferior MI is common and usually transient; Mobitz II in anterior MI indicates extensive septal necrosis
Atropine may be used acutely for symptomatic bradycardia but is less effective in infra-nodal (Mobitz II) block
Overview
Key Facts
Second degree heart block describes intermittent failure of conduction from the atria to the ventricles. It is classified into Mobitz type I (Wenckebach) and Mobitz type II, which have significantly different clinical implications and management.
Epidemiology
Mobitz type I: relatively common, especially in young/athletic individuals and during sleep
Mobitz type II: less common, typically occurs in older patients with structural heart disease
Mobitz II accounts for ~20-30% of second-degree blocks
Aetiology
Mobitz Type I:
Increased vagal tone (athletes, sleep)
Drugs: beta-blockers, CCBs, digoxin
Inferior MI (transient AV nodal ischaemia)
Myocarditis, rheumatic fever
Mobitz Type II:
Degenerative conduction system disease (Lenegre/Lev disease)
Anterior MI (septal branch of LAD supplies His-Purkinje)
Post-cardiac surgery
Infiltrative: sarcoidosis, amyloidosis
Aortic valve calcification extending to conduction system
Pathophysiology
Mobitz I (Wenckebach):
Block occurs at the level of the AV node
Incremental delay in AV conduction until an impulse fails entirely
QRS is usually narrow (unless pre-existing bundle branch block)
Mobitz II:
Block occurs below the AV node (His bundle or bundle branches)
Intermittent complete failure of infra-nodal conduction
QRS is often wide (reflecting bilateral bundle branch disease)
Higher risk of sudden progression to complete heart block
Complete failure of conduction between atria and ventricles (third-degree AV block) with independent atrial and ventricular rhythms. Requires urgent pacing in most acquired cases.
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Key Facts
Complete AV dissociation: P waves and QRS complexes are independent with no relationship between them
Common causes: degenerative conduction disease (most common in elderly), inferior/anterior MI, post-cardiac surgery, drugs
Broad QRS escape rhythm carries higher risk of asystole and is more haemodynamically unstable
Treatment: permanent pacemaker is definitive treatment for symptomatic or acquired complete heart block
Atropine may be used acutely but is often ineffective in infra-nodal block; isoprenaline or temporary pacing preferred
Congenital CHB: associated with maternal anti-Ro/La antibodies (neonatal lupus) and structural heart defects
Post-inferior MI: may be transient; post-anterior MI indicates extensive septal necrosis with poor prognosis
Overview
Key Facts
Complete heart block (third-degree AV block) is characterised by complete absence of conduction between the atria and ventricles. The atria and ventricles beat independently, with the ventricles relying on an escape rhythm at a rate slower than the sinus rate.
Epidemiology
Incidence increases with age: rare in young adults, common in elderly
Leading indication for permanent pacemaker implantation worldwide
Congenital CHB: incidence ~1 in 15,000-20,000 live births
Accounts for ~5-10% of acute MI cases with conduction disturbance
Aetiology
Acquired (most common):
Degenerative: idiopathic fibrosis of the conduction system (Lenegre/Lev disease) - commonest cause in elderly
Ischaemic: inferior MI (usually transient, AV nodal), anterior MI (usually permanent, infra-nodal)
Dysfunction of the sinoatrial node causing a combination of inappropriate sinus bradycardia, sinus pauses/arrest, and alternating tachycardia-bradycardia. Most common indication for pacemaker in elderly.
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Key Facts
Also known as sinus node dysfunction; encompasses sinus bradycardia, sinus arrest/pauses, sinoatrial exit block, and tachy-brady syndrome
Tachy-brady syndrome: alternating episodes of tachycardia (usually atrial fibrillation) and bradycardia
Most common in elderly due to age-related fibrosis of the SA node
Definitive treatment: permanent pacemaker (atrial-based pacing: AAI or DDD preferred over VVI to reduce AF risk)
Drug challenge: antiarrhythmic drugs for tachycardia episodes may worsen bradycardia - pacemaker often needed first
Prevalence: increases significantly with age; most common indication for pacemaker implantation
Overview
Key Facts
Sick sinus syndrome (SSS) or sinus node dysfunction encompasses a spectrum of SA node abnormalities including inappropriate sinus bradycardia, sinus pauses or arrest, sinoatrial exit block, chronotropic incompetence, and the tachy-brady syndrome.
Epidemiology
Predominantly affects the elderly (mean age at diagnosis ~65-70 years)
Prevalence: ~1 in 600 patients over 65 years
Most common indication for permanent pacemaker implantation
No significant sex difference
Aetiology
Idiopathic degenerative fibrosis of the SA node (most common)
Conduction delay or block in one of the bundle branches causing widened QRS (≥120 ms) with characteristic morphology. LBBB may indicate structural heart disease; RBBB can be a normal variant.
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Key Facts
QRS duration ≥120 ms with characteristic morphology distinguishes complete BBB from incomplete (<120 ms)
RBBB: RSR' pattern in V1-V2 ('M' shaped), wide slurred S wave in V1, I, V6; may be normal variant in young/healthy individuals
LBBB: broad notched R wave in I, aVL, V5-V6 ('M' shaped), deep S wave in V1-V3; usually indicates structural heart disease
New LBBB with chest pain should be treated as STEMI equivalent and activate PPCI pathway
LBBB makes interpretation of ST segments unreliable - use Sgarbossa criteria for MI diagnosis with LBBB
Bifascicular block (RBBB + left anterior or posterior hemiblock): risk of progression to CHB if acute
LBBB is associated with increased cardiovascular mortality; RBBB in isolation is generally benign
Overview
Key Facts
Bundle branch block (BBB) occurs when conduction is delayed or blocked in one of the main bundle branches of the His-Purkinje system. This produces a wide QRS complex (≥120 ms) with a characteristic morphology depending on whether the right or left bundle is affected.
Epidemiology
RBBB: prevalence ~0.8-2% in the general population; increases with age
LBBB: prevalence ~0.5-1%; strongly associated with age and structural heart disease
RBBB is more common than LBBB in the general population
LBBB prevalence: ~5-10% in patients with heart failure
Aetiology
RBBB:
Normal variant (especially in young adults)
Right heart strain: PE, cor pulmonale, pulmonary hypertension
Ischaemic heart disease
Atrial septal defect (ASD)
Myocarditis, cardiomyopathy
Post-cardiac surgery, right heart catheterisation
LBBB:
Hypertensive heart disease
Ischaemic heart disease
Dilated cardiomyopathy
Aortic valve disease (especially aortic stenosis)
Degenerative conduction disease
Myocarditis
Post-TAVI (up to 20-30%)
Pathophysiology
Block in one bundle branch causes the ventricle on that side to depolarise late via slow cell-to-cell conduction from the contralateral ventricle
This produces a widened QRS with a characteristic 'double-peak' pattern
Dyssynchronous ventricular contraction in LBBB can impair cardiac output and contribute to heart failure
LBBB can be an indication for cardiac resynchronisation therapy (CRT) in heart failure
Clinical Presentation
RBBB
Usually asymptomatic if isolated
Wide splitting of S2 (delayed closure of pulmonary valve)
May be associated with symptoms of underlying condition (e.g., PE, ASD)
LBBB
May be asymptomatic or associated with symptoms of underlying cardiac disease
Reversed splitting of S2 (delayed closure of aortic valve)
New LBBB with acute chest pain = STEMI equivalent
Chronic LBBB may contribute to heart failure via dyssynchrony
Red Flags
New LBBB with chest pain: treat as STEMI - activate PPCI pathway
New BBB following syncope: risk of intermittent higher-degree block
Alternating RBBB and LBBB: indicates bilateral bundle disease, high risk of CHB
Bifascicular block with syncope: consider pacemaker
Differential Diagnosis
Diagnosis
Key Features
Investigation
Ventricular pre-excitation (WPW)
Short PR, delta wave, wide QRS
ECG
Ventricular paced rhythm
Pacing spikes, LBBB morphology (RV pacing)
Device check
Hyperkalaemia
Broad QRS, peaked T waves
U&Es
Ventricular tachycardia
Wide complex tachycardia, AV dissociation
ECG, clinical context
Incomplete BBB
QRS 100-119 ms with BBB morphology
ECG
Na-channel blocker toxicity
Wide QRS, history of TCA/flecainide overdose
Drug history, levels
Diagnosis / Investigation
Bedside
12-lead ECG: QRS morphology and width, axis, Sgarbossa criteria if LBBB
Comparison with previous ECGs: to determine if BBB is new or old
Pre-excitation syndrome caused by an accessory pathway (bundle of Kent) between atria and ventricles, characterised by short PR interval, delta wave, and risk of re-entrant tachycardias.
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Accessory pathway (bundle of Kent) bypasses the AV node allowing pre-excitation of the ventricle
Orthodromic AVRT (narrow complex): most common tachycardia (~95%); anterograde conduction via AV node, retrograde via accessory pathway
AF with WPW is DANGEROUS: rapid conduction via accessory pathway can cause VF - AVOID AV nodal blockers (adenosine, verapamil, digoxin, beta-blockers)
AF with WPW treatment: IV flecainide or DC cardioversion if haemodynamically unstable
Definitive treatment: catheter ablation of the accessory pathway (success rate >95%)
Prevalence: ~1-3 per 1,000 population; associated with Ebstein anomaly and hypertrophic cardiomyopathy
Risk of sudden death: ~0.1-0.5% per year in symptomatic patients; accessory pathway ERP <250 ms is high risk
Overview
Key Facts
Wolff-Parkinson-White (WPW) syndrome is a pre-excitation condition caused by an accessory electrical pathway (bundle of Kent) connecting the atria and ventricles, bypassing the AV node. This allows early ventricular activation producing the characteristic delta wave and predisposes to re-entrant tachycardias.
Epidemiology
Prevalence: 1-3 per 1,000 population
More common in males (~60-70%)
Usually presents in adolescence or young adulthood (20-40 years)
50-60% of patients with WPW pattern experience symptomatic tachycardia
Risk of sudden cardiac death: ~0.1-0.5% per year
Aetiology
Congenital accessory pathway persisting from embryological development
Most cases sporadic; rarely familial (associated with PRKAG2 mutations)
Inherited cardiac channelopathy characterised by a distinctive coved ST elevation in V1-V3 with risk of ventricular fibrillation and sudden cardiac death, typically in young men.
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Key Facts
Type 1 Brugada pattern: coved ST elevation ≥2 mm in ≥1 right precordial lead (V1-V3) followed by negative T wave - only diagnostic pattern
Autosomal dominant inheritance with incomplete penetrance; SCN5A gene mutation in ~20-25% of cases
Most common cause of sudden cardiac death in young men in Southeast Asian populations
ICD implantation is the only proven treatment to prevent sudden cardiac death
Ajmaline/flecainide provocation test: used to unmask concealed Brugada pattern (types 2/3 → type 1 conversion)
Avoid: class I antiarrhythmics, fever (treat aggressively with paracetamol), and drugs on Brugada drug list
Quinidine: only drug shown to suppress arrhythmias in Brugada (used as adjunct to ICD or when ICD refused)
Overview
Key Facts
Brugada syndrome is a genetic cardiac channelopathy associated with a characteristic ECG pattern and a risk of ventricular fibrillation (VF) and sudden cardiac death (SCD), typically occurring at rest or during sleep. It is an important cause of sudden unexplained death, particularly in young men.
Epidemiology
Prevalence: 1-5 per 10,000 worldwide; higher in Southeast Asia (up to 1 in 1,000)
Male:female ratio 8-10:1 for clinical events (testosterone modulates ion channel expression)
Mean age at diagnosis: 40 years
Accounts for ~4% of all sudden cardiac deaths and ~20% of SCD in structurally normal hearts
Aetiology
SCN5A (sodium channel gene) mutations identified in ~20-25%
Multiple other genes identified (SCN1B, CACNA1C, CACNB2, KCNE3, etc.)
Many cases are genetically unresolved
Autosomal dominant with variable expressivity and incomplete penetrance
Pathophysiology
Loss-of-function mutations in cardiac sodium channels (Nav1.5) reduce sodium current (INa)
This creates transmural voltage gradient in the right ventricular outflow tract (RVOT) between epicardium and endocardium
The gradient produces the characteristic coved ST elevation and predisposes to phase 2 re-entry, triggering VF
Arrhythmias typically occur at rest or during sleep when vagal tone is high
Clinical Presentation
Typical Presentation
Syncope or cardiac arrest (VF), typically during rest or sleep
Family history of sudden cardiac death (especially young males)
Palpitations
Nocturnal agonal respirations (witnessed by partner)
ECG Patterns
Type 1 (diagnostic): ≥2 mm coved ST elevation in V1-V3, followed by negative T wave
Type 2 (saddle-back): ≥2 mm ST elevation with ≥1 mm concave ST, positive or biphasic T wave - NOT diagnostic alone
Type 3: <1 mm ST elevation - NOT diagnostic
ECG pattern may be intermittent and provoked by sodium channel blockers, fever, or vagal activity
Triggers for Arrhythmia
Fever (important - treat aggressively)
Excessive alcohol
Large meals (vagal stimulation)
Sleep/rest
Drugs: class I antiarrhythmics, TCAs, lithium, cocaine
Inherited or acquired disorder of cardiac repolarisation characterised by prolonged QTc interval, predisposing to Torsades de Pointes, syncope, and sudden cardiac death.
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Key Facts
Congenital prevalence: ~1 in 2,000; Romano-Ward (autosomal dominant, isolated cardiac) most common; Jervell-Lange-Nielsen (autosomal recessive + deafness)
LQT1 (KCNQ1): triggered by exercise (especially swimming); LQT2 (KCNH2): triggered by auditory stimuli/emotional stress; LQT3 (SCN5A): events at rest/sleep
QTc >500 ms is high risk for Torsades de Pointes and sudden death
ICD indicated for cardiac arrest survivors or recurrent syncope despite beta-blocker therapy
Left cardiac sympathetic denervation for patients with recurrent events on beta-blockers who are not candidates for or decline ICD
Acquired LQTS: drugs (see QT-prolonging drug lists), hypokalaemia, hypomagnesaemia, hypothyroidism
Schwartz score used for clinical diagnosis of congenital LQTS (≥3.5 = high probability)
Overview
Key Facts
Long QT syndrome (LQTS) is a disorder of myocardial repolarisation characterised by a prolonged QT interval on the ECG, predisposing to polymorphic VT (Torsades de Pointes), syncope, and sudden cardiac death. It may be congenital (inherited channelopathy) or acquired (drugs, electrolytes).
Epidemiology
Congenital LQTS: estimated prevalence 1 in 2,000
Acquired LQTS: far more common, primarily drug-induced
Women have a longer baseline QTc and are at higher risk of drug-induced TdP
Leading cause of sudden cardiac death in young people with structurally normal hearts
Aetiology
Congenital:
LQT1 (KCNQ1 - IKs channel): ~40% of cases; triggered by exercise/swimming
LQT2 (KCNH2/HERG - IKr channel): ~30% of cases; triggered by sudden auditory stimuli
LQT3 (SCN5A - sodium channel gain-of-function): ~10%; events at rest/sleep
Inherited arrhythmia syndrome characterised by exercise or emotion-triggered bidirectional or polymorphic ventricular tachycardia in the absence of structural heart disease or QT prolongation.
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Key Facts
Resting ECG is normal - diagnosis requires exercise stress testing which provokes bidirectional or polymorphic VT
Bidirectional VT: alternating QRS axis on a beat-by-beat basis - highly characteristic of CPVT
RYR2 gene mutation (autosomal dominant) accounts for ~60% of cases; CASQ2 (autosomal recessive) ~5%
Beta-blockers (nadolol preferred) are first-line treatment - reduce events significantly
Flecainide (100-150mg BD): important adjunct therapy; specifically suppresses RYR2-mediated arrhythmias
ICD for cardiac arrest survivors; however, ICD shocks can trigger catecholamine surges and arrhythmia storms
Onset: typically in childhood or adolescence (mean age 7-12 years); syncope during exercise/emotion
Mortality: untreated CPVT has ~30% cardiac event rate by age 30
Overview
Key Facts
Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an inherited arrhythmia syndrome characterised by adrenergically-mediated polymorphic or bidirectional ventricular tachycardia in the presence of a structurally normal heart and a normal resting ECG. It is a potent cause of sudden cardiac death in children and young adults.
Epidemiology
Estimated prevalence: 1 in 10,000
Onset typically in childhood: mean age at first event ~7-12 years
Males and females equally affected
Accounts for ~12% of autopsy-negative sudden cardiac death in the young
Aetiology
RYR2 (ryanodine receptor 2): autosomal dominant, ~60% of cases
CASQ2 (calsequestrin 2): autosomal recessive, ~5% of cases
Other rare genes: TRDN, CALM1, CALM2, CALM3
~25% genetically unresolved
Pathophysiology
Mutations cause abnormal calcium release from the sarcoplasmic reticulum during adrenergic stimulation
Progressive narrowing of the aortic valve orifice causing left ventricular outflow obstruction. Most commonly caused by degenerative calcification in the elderly or bicuspid aortic valve in younger patients.
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Key Facts
Classic triad: exertional angina, syncope, and heart failure - onset of symptoms indicates poor prognosis without intervention
Murmur: ejection systolic murmur, best heard at right upper sternal edge, radiating to carotids; slow-rising pulse
Severe AS criteria: valve area <1.0 cm², mean gradient >40 mmHg, peak velocity >4 m/s
Aortic valve replacement (surgical AVR or TAVI) is the definitive treatment for symptomatic severe AS
TAVI (transcatheter aortic valve implantation) is standard for inoperable/high-risk patients and increasingly used in intermediate/low risk
Prognosis without intervention: median survival ~2-3 years from symptom onset (angina 5 yrs, syncope 3 yrs, heart failure 2 yrs)
Most common valvular heart disease in the developed world; prevalence ~3% over age 75
NICE NG208 recommends echocardiography for all patients with a cardiac murmur and symptoms
Overview
Key Facts
Aortic stenosis (AS) is the most common acquired valvular heart disease in developed countries. It involves progressive narrowing and calcification of the aortic valve, leading to obstruction of left ventricular outflow.
Epidemiology
Prevalence: ~2-3% in adults >65 years; ~5% in >75 years
Most common indication for valve replacement surgery
Male:female ratio ~2:1 for calcific AS
Bicuspid aortic valve affects ~1-2% of the population
Aetiology
Degenerative calcification (senile/calcific AS): most common in developed countries (>65 years)
Bicuspid aortic valve: most common cause in <65 years; presents 10-20 years earlier than tricuspid valve AS
Rheumatic heart disease: common in developing countries; commissural fusion
Retrograde blood flow from the aorta into the left ventricle during diastole due to incompetent aortic valve closure. Causes include bicuspid valve, aortic root dilatation, and infective endocarditis.
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Key Facts
Murmur: early diastolic decrescendo murmur, best heard at left sternal edge with patient sitting forward and in expiration
Acute AR (endocarditis, dissection): medical emergency with pulmonary oedema and cardiogenic shock - requires urgent surgery
Chronic AR: well-tolerated for years due to LV dilatation and eccentric hypertrophy; symptoms late
Medical therapy: vasodilators (nifedipine, ACEi) may be used as bridge or if surgery declined; no proven delay in surgery
Overview
Key Facts
Aortic regurgitation (AR) is the retrograde flow of blood from the aorta back into the left ventricle during diastole due to failure of the aortic valve to close properly. It may be acute (surgical emergency) or chronic (long compensated period before symptoms develop).
Epidemiology
Prevalence of moderate-severe AR: ~0.5-1% in population-based studies
More common in men (~75%)
Incidence increases with age
Bicuspid aortic valve: most common cause in younger patients
Narrowing of the mitral valve orifice obstructing blood flow from the left atrium to the left ventricle. Almost exclusively caused by rheumatic heart disease in the UK.
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Key Facts
Rheumatic heart disease is the cause in >95% of cases worldwide; declining incidence in the UK
Murmur: low-pitched mid-diastolic rumble at apex, best heard in left lateral decubitus with bell; opening snap may be present
Atrial fibrillation develops in ~40-50% due to left atrial dilatation; high stroke risk requiring anticoagulation
Severe MS: valve area <1.0 cm² (normal 4-6 cm²); mean gradient >10 mmHg
Symptoms precipitated by increased heart rate (exercise, fever, pregnancy, AF) - reduces diastolic filling time
NICE NG208: intervention for symptomatic severe MS or asymptomatic with pulmonary hypertension
Percutaneous mitral balloon valvuloplasty (PMBV): treatment of choice if anatomy suitable (Wilkins score ≤8)
Malar flush: dusky purple discolouration of cheeks from chronic low cardiac output and pulmonary hypertension
Overview
Key Facts
Mitral stenosis (MS) is narrowing of the mitral valve orifice that impedes diastolic blood flow from the left atrium to the left ventricle. It is almost exclusively caused by rheumatic heart disease.
Epidemiology
Declining incidence in developed countries; still prevalent in developing nations
Rheumatic MS: predominantly affects women (F:M ~2:1)
Symptoms typically develop 20-40 years after rheumatic fever episode
Prevalence in UK: ~1-2 per 1,000 adults (mostly immigrant populations)
Percutaneous mitral balloon valvuloplasty (PMBV): treatment of choice for symptomatic severe MS with favourable anatomy (Wilkins score ≤8), sinus rhythm or AF, no LA thrombus, and no more than mild MR
Mitral valve replacement (MVR): if anatomy unfavourable for PMBV, significant MR, or failed PMBV
Mechanical: requires lifelong warfarin
Bioprosthetic: limited durability
Closed mitral valvotomy: historical; rarely performed now
Referral Criteria
Symptomatic severe MS: cardiology referral for intervention assessment
New AF in MS: rate control and anticoagulation initiation
Pregnancy with MS: specialist obstetric/cardiology joint care
Systemic embolism: urgent evaluation
Prognosis
Untreated symptomatic severe MS: 10-year survival ~50-60%
Post-PMBV: good results with event-free survival ~60-70% at 10 years
Post-MVR: 10-year survival ~70%
AF in MS: significantly increases embolic risk (~5-15% per year without anticoagulation)
Pregnancy with severe MS: high risk of maternal complications (pulmonary oedema, AF)
Retrograde blood flow from the left ventricle to the left atrium during systole due to incompetent mitral valve closure. The most common valvular lesion in developed countries.
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Key Facts
Most common valvular lesion in developed countries; degenerative (myxomatous/MVP) is the leading cause
Murmur: pansystolic at apex, radiating to axilla; soft S1, widely split S2, S3 in severe MR
Acute severe MR (chordal/papillary rupture, endocarditis): medical emergency; emergency surgery often required
Surgical indications (NICE NG208): symptomatic severe MR, or asymptomatic with LVEF ≤60% or LVESD ≥40 mm
Mitral valve repair preferred over replacement when feasible (better outcomes, avoids anticoagulation)
MitraClip (percutaneous edge-to-edge repair): option for high-risk surgical patients
Overview
Key Facts
Mitral regurgitation (MR) is the backward flow of blood from the left ventricle into the left atrium during systole through an incompetent mitral valve. It is classified as primary (intrinsic valve disease) or secondary/functional (due to LV remodelling).
Epidemiology
Most common valvular lesion: affects ~2-3% of the population
Prevalence increases significantly with age
Degenerative MR is the most common cause in developed countries
Secondary MR occurs in ~30-50% of patients with heart failure and reduced EF
Aetiology
Primary MR:
Myxomatous degeneration/mitral valve prolapse (most common in developed countries)
Displacement of one or both mitral valve leaflets into the left atrium during systole by ≥2 mm. The most common valve abnormality, usually benign but may cause significant mitral regurgitation.
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Key Facts
Most common valvular abnormality: prevalence ~2-3% of the population
Auscultation: mid-systolic click ± late systolic murmur; click moves earlier with standing/Valsalva and later with squatting
Usually benign with excellent prognosis; ~5-10% develop significant mitral regurgitation over decades
Myxomatous degeneration of mitral valve leaflets and chordae is the underlying pathology
Surgery: mitral valve repair (not replacement) is preferred for severe MR from MVP
NICE NG208: echocardiographic surveillance for those with moderate or more MR
Overview
Key Facts
Mitral valve prolapse (MVP) is the billowing of one or both mitral valve leaflets into the left atrium during systole by ≥2 mm beyond the mitral annular plane. It is the most common valvular abnormality and is usually benign.
Epidemiology
Prevalence: ~2-3% of the general population
More commonly diagnosed in women, but men more likely to develop significant MR
Peak diagnosis in young adulthood (20-40 years)
Most common cause of isolated mitral regurgitation requiring surgery in developed countries
Aetiology
Primary (myxomatous): most common; redundant leaflet tissue with myxomatous degeneration
Often idiopathic or familial (autosomal dominant with variable penetrance)
FLNA gene mutations in some X-linked familial cases
Secondary: associated with connective tissue disorders
Auscultation: mid-systolic click ± late systolic murmur with dynamic manoeuvres
ECG: usually normal; may show T-wave inversions in inferior leads, PVCs
Bloods
Routine bloods generally normal
BNP/NT-proBNP: if heart failure suspected
Imaging
Transthoracic echocardiography: diagnostic gold standard
Leaflet displacement ≥2 mm beyond annular plane in parasternal long-axis view
Leaflet thickness ≥5 mm (myxomatous/Barlow disease)
Assess degree of MR, LV function and dimensions
TOE: detailed valve anatomy pre-surgical repair
Cardiac MRI: if echo equivocal; late gadolinium enhancement in papillary muscles may identify arrhythmic MVP
Special Tests
Holter monitor: if palpitations; assess for ventricular ectopy
Exercise testing: if symptoms disproportionate to echo findings
Genetic testing: if Marfan or other connective tissue disorder suspected
Management
Non-pharmacological
Reassurance for asymptomatic patients with trivial/mild MR
No exercise restriction for mild MVP without significant MR
Avoid excessive caffeine, stimulants if palpitations bothersome
Serial echocardiographic monitoring: annually if moderate MR; 3-5 yearly if mild
Pharmacological
Beta-blockers (e.g., propranolol 10-40mg TDS): for symptomatic palpitations/chest pain
Standard heart failure medications if significant MR develops (ACEi, beta-blocker, diuretics)
Antibiotic prophylaxis: not routinely recommended by NICE for dental procedures (only if prior endocarditis)
Antiplatelet therapy for unexplained TIA/stroke (aspirin 75mg or clopidogrel 75mg)
Surgical/Interventional
Mitral valve repair: preferred for severe MR from MVP (repair rates >90% in experienced centres)
Indications: same as for primary MR (symptomatic severe MR; asymptomatic with LVEF ≤60% or LVESD ≥40 mm; flail leaflet; new AF; pulmonary hypertension)
Emergency surgery: for acute chordal rupture with severe MR and haemodynamic compromise
Referral Criteria
Moderate or greater MR: cardiology follow-up for serial monitoring
Severe MR: surgical assessment
Symptoms disproportionate to echo findings: further investigation
Marfanoid features: genetics referral
Prognosis
Excellent prognosis for the majority: same life expectancy as general population
~5-10% develop significant MR requiring intervention over 10-20 years
Risk of progression: older age, male sex, thickened leaflets (>5 mm), flail leaflet
Risk of endocarditis: ~1-2% lifetime if no significant MR; higher with MR
Sudden cardiac death: extremely rare (<1 in 10,000 per year); associated with bileaflet prolapse, complex ventricular ectopy, fibrotic papillary muscles
Post-mitral valve repair for MVP: excellent long-term outcomes with >90% freedom from reoperation at 10 years
Backward flow of blood from the right ventricle into the right atrium during systole. Most commonly secondary (functional) due to RV dilatation from pulmonary hypertension or left-sided heart disease.
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Key Facts
Most commonly functional/secondary (~85%): RV dilatation from pulmonary hypertension, left-sided heart disease, or RV infarction
Murmur: pansystolic at left lower sternal edge, increases with inspiration (Carvallo sign)
Signs of right heart failure: elevated JVP with prominent CV waves, hepatomegaly (pulsatile), peripheral oedema, ascites
Treatment: primarily directed at the underlying cause; diuretics for congestion
Surgery: tricuspid annuloplasty at time of left-sided valve surgery if significant TR; isolated TV surgery less common
Overview
Key Facts
Tricuspid regurgitation (TR) is the backward flow of blood from the right ventricle to the right atrium during systole. Mild TR is extremely common and often physiological. Significant TR is most often secondary to right ventricular dilatation.
Epidemiology
Mild/trace TR: detected in up to 70-80% of normal echocardiograms
Clinically significant TR: prevalence ~0.5-1%
Functional TR accounts for ~85% of significant cases
Increasing prevalence with age and in patients with left-sided heart disease
Obstruction to right ventricular outflow at the level of the pulmonary valve, most commonly congenital. Usually well tolerated; severe cases may require balloon valvuloplasty.
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Key Facts
Most commonly congenital - accounts for ~10% of congenital heart disease
Murmur: ejection systolic murmur at left upper sternal edge, radiating to the back; widely split S2 with soft P2
Mild-moderate PS is usually asymptomatic with excellent prognosis and requires no intervention
Severe PS: RV peak gradient >60 mmHg - symptoms of exertional dyspnoea, fatigue, syncope, right heart failure
Percutaneous balloon valvuloplasty: treatment of choice for severe valvular PS (success rate >90%)
Pulmonary stenosis (PS) is obstruction to right ventricular outflow, most commonly at the valvular level. It is predominantly congenital and accounts for approximately 10% of all congenital heart defects.
Epidemiology
~10% of all congenital heart disease
Valvular PS is most common form (~90%)
Equally affects males and females
Usually diagnosed in childhood or incidentally in adulthood
Aetiology
Congenital valvular PS (most common): dome-shaped valve with fused commissures
Dysplastic valve: thickened, immobile leaflets (associated with Noonan syndrome - less responsive to balloon valvuloplasty)
Infundibular (subvalvular): muscular obstruction in RVOT
Supravalvular: pulmonary artery stenosis (associated with Williams syndrome, Alagille syndrome, rubella)
Infection of the endocardial surface of the heart, typically involving heart valves, with formation of vegetations. A serious condition with significant morbidity and mortality requiring prolonged antibiotics and often surgery.
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Key Facts
Modified Duke criteria used for diagnosis: major criteria (positive blood cultures, positive echo) and minor criteria
Most common organisms: Staphylococcus aureus (most common overall and in acute IE), viridans group streptococci (subacute native valve IE)
3 sets of blood cultures from different sites before starting antibiotics (ideally within 12 hours)
Empirical treatment (native valve): amoxicillin 2g IV QDS + gentamicin (NICE/BNF); if penicillin-allergic or prosthetic valve: vancomycin-based regimen
Surgery indicated for: heart failure from valve destruction, uncontrolled infection, large vegetations (>10 mm) with embolic risk, prosthetic valve endocarditis
Infective endocarditis (IE) is an infection of the endocardial surface of the heart, most commonly involving the heart valves. Vegetations composed of platelets, fibrin, microorganisms, and inflammatory cells form on damaged endocardium.
Epidemiology
Incidence: ~3-10 per 100,000 per year in the UK
Increasing incidence due to: ageing population, prosthetic valves, cardiac devices, IV drug use, healthcare-associated infections
Male:female ratio ~2:1
Median age: 50-60 years (increasing)
Aetiology
Organisms:
Staphylococcus aureus: most common overall; acute presentation; IVDU-associated
Viridans group streptococci (S. mitis, S. sanguinis): subacute native valve IE; dental origin
Chronic valvular heart disease resulting from acute rheumatic fever following Group A streptococcal pharyngitis. Mitral valve is most commonly affected. Declining in UK but still prevalent globally.
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Key Facts
Sequela of acute rheumatic fever (ARF) triggered by Group A beta-haemolytic streptococcus (GAS) pharyngitis
Molecular mimicry: streptococcal M protein antigens cross-react with cardiac myosin and valve tissue
Jones criteria for ARF diagnosis: major (carditis, polyarthritis, chorea, erythema marginatum, subcutaneous nodules) + evidence of streptococcal infection
Mitral valve most commonly affected (~95%); mitral stenosis is the classic chronic lesion
Secondary prophylaxis: phenoxymethylpenicillin 250mg BD (or IM benzathine penicillin monthly) for ≥10 years or until age 40
Leading cause of acquired heart disease in children and young adults in developing countries
UK prevalence: low and declining, primarily in immigrant populations
Overview
Key Facts
Rheumatic heart disease (RHD) is the chronic cardiac manifestation of acute rheumatic fever (ARF), an autoimmune inflammatory condition triggered by Group A streptococcal (GAS) pharyngitis. It results in permanent valvular damage, most commonly mitral stenosis.
Epidemiology
~33 million people affected worldwide; >300,000 deaths annually
Predominantly affects children and young adults (5-15 years for ARF)
Rare in UK (<1 per 100,000); common in developing countries, Aboriginal populations, Pacific Islanders
UK cases predominantly in immigrant populations from endemic areas
Aetiology
Acute rheumatic fever: autoimmune response 2-4 weeks after untreated GAS pharyngitis
Molecular mimicry: antibodies against streptococcal M protein cross-react with cardiac myosin, valvular glycoproteins, and other tissues
Recurrent episodes of ARF cause cumulative valve damage
Inflammation of the pericardium causing characteristic sharp chest pain relieved by sitting forward, with widespread saddle-shaped ST elevation on ECG. Most cases are idiopathic/viral and self-limiting.
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Key Facts
Diagnosis requires ≥2 of 4 criteria: typical chest pain, pericardial friction rub, widespread saddle-shaped ST elevation with PR depression on ECG, new/worsening pericardial effusion
Most common cause: idiopathic/viral (~80-90% in developed countries); Coxsackie, echovirus, adenovirus, EBV
Treatment: NSAIDs (ibuprofen 600mg TDS for 1-2 weeks, tapering) + colchicine 500mcg BD for 3 months (COPE trial)
Colchicine reduces recurrence rate from ~30% to ~15% (COPE and CORE trials)
Avoid anticoagulants if possible - risk of haemopericardium/tamponade
Exercise restriction: avoid strenuous exercise until symptoms resolve and inflammatory markers normalise
Recurrent pericarditis: occurs in ~30%; colchicine reduces recurrence; consider low-dose corticosteroids or anakinra for refractory cases
Important differential from STEMI: ST elevation is diffuse/widespread, concave upwards, with PR depression; no reciprocal changes
Overview
Key Facts
Acute pericarditis is inflammation of the pericardial layers (visceral and parietal pericardium). It is the most common pericardial disease encountered in clinical practice, presenting with chest pain, friction rub, and characteristic ECG changes.
Epidemiology
Accounts for ~5% of emergency chest pain presentations
12-lead ECG: widespread concave-upward (saddle-shaped) ST elevation in most leads; PR depression (especially lead II); reciprocal ST depression and PR elevation in aVR
Stage 1: diffuse ST elevation + PR depression
Stage 2: ST normalises, T-wave flattening
Stage 3: diffuse T-wave inversion
Stage 4: normalisation
Observations: temperature, HR, BP, SpO₂
Bloods
CRP/ESR: elevated (diagnostic criterion and guides duration of treatment)
FBC: leucocytosis (may be normal in viral)
Troponin: elevated in myopericarditis (~30%); indicates myocardial involvement
U&Es: uraemia
TFTs: hypothyroidism
ANA, anti-dsDNA: if autoimmune cause suspected
Blood cultures: if purulent pericarditis suspected
Imaging
Echocardiography: pericardial effusion (may be absent in dry pericarditis); assess for tamponade features
CXR: may be normal; enlarged cardiac silhouette if large effusion (flask-shaped heart)
Cardiac MRI: confirms pericardial inflammation (late gadolinium enhancement of pericardium); differentiates from myocarditis
Life-threatening compression of the heart by fluid accumulation in the pericardial space, impairing diastolic filling and reducing cardiac output. Requires emergency pericardiocentesis.
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Beck triad: hypotension, raised JVP, muffled heart sounds - classic but found in only ~30% of cases
Pulsus paradoxus: >10 mmHg fall in systolic BP during inspiration - highly suggestive
Echocardiography is the diagnostic investigation of choice: pericardial effusion with RA/RV diastolic collapse, IVC plethora
Emergency pericardiocentesis is the definitive acute treatment - needle aspiration via subxiphoid approach
Rapid accumulation of even small volumes (100-200 mL) can cause tamponade; slow accumulation allows pericardial stretch (up to 1-2 L)
Avoid diuretics and vasodilators - worsen haemodynamic compromise; give IV fluids to maintain preload
Electrical alternans on ECG: alternating QRS amplitude - due to swinging heart in large effusion
Overview
Key Facts
Cardiac tamponade occurs when fluid accumulation in the pericardial space reaches a pressure that impairs cardiac filling, leading to haemodynamic compromise. It is a medical emergency requiring urgent drainage.
Epidemiology
Incidence depends on underlying cause; occurs in ~2% of penetrating chest trauma
Malignancy is the most common cause of large pericardial effusion/tamponade in developed countries
Can occur with any cause of pericardial effusion
Aetiology
Malignancy: lung, breast, lymphoma, melanoma (most common in chronic effusions)
Chronic fibrotic thickening and calcification of the pericardium leading to impaired diastolic filling and progressive right heart failure. Often presents insidiously with ascites and oedema.
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Thickened, fibrotic, often calcified pericardium encases the heart and restricts diastolic filling
Kussmaul sign: paradoxical rise in JVP during inspiration - characteristic (also seen in RV infarction)
Pericardial knock: early diastolic sound from abrupt cessation of ventricular filling
Differentiation from restrictive cardiomyopathy is a classic exam question - MRI, CT, and catheterisation help distinguish
Definitive treatment: pericardiectomy (surgical stripping of pericardium); medical therapy is supportive only
CT/MRI show pericardial thickening >4 mm ± calcification; cardiac catheterisation shows equalisation of diastolic pressures
Overview
Key Facts
Constrictive pericarditis (CP) is a chronic condition in which the pericardium becomes thickened, fibrotic, and sometimes calcified, encasing the heart in a rigid shell that impairs diastolic filling. This leads to signs of right (and eventually biventricular) heart failure.
Epidemiology
Uncommon in developed countries; more common where TB is prevalent
Post-cardiac surgery and idiopathic are the leading causes in developed countries
May develop months to years after the initial pericardial insult
Occurs in ~1% of acute viral pericarditis cases; higher with TB/purulent/radiation pericarditis
Aetiology
TB pericarditis: most common cause worldwide
Idiopathic/post-viral: most common in developed countries
Post-cardiac surgery: especially CABG
Post-radiation: mediastinal radiotherapy (Hodgkin lymphoma, breast cancer)
Inflammation of the myocardium, most commonly viral, presenting with chest pain, heart failure, or arrhythmias. Cardiac MRI is the gold standard non-invasive investigation.
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Most common cause: viral - Coxsackie B, parvovirus B19, adenovirus, HHV-6, SARS-CoV-2
Cardiac MRI is the gold standard non-invasive diagnostic tool: T1/T2 mapping, late gadolinium enhancement (LGE) in mid-wall or subepicardial pattern
Endomyocardial biopsy (EMB): gold standard for definitive histological diagnosis but rarely performed due to sampling error and risk
Presentation: mimics ACS with chest pain and troponin rise but normal coronary arteries on angiography
Fulminant myocarditis: acute onset with severe LV dysfunction and cardiogenic shock - requires intensive support
Prognosis: most recover fully; ~20-30% develop dilated cardiomyopathy; fulminant myocarditis paradoxically has better long-term outcomes if patient survives
Overview
Key Facts
Myocarditis is inflammation of the myocardium characterised by myocyte necrosis and inflammatory infiltrate. It has variable presentation from subclinical disease to fulminant heart failure and sudden death.
Epidemiology
True incidence unknown: estimated 10-22 cases per 100,000 per year
More common in young adults, especially males (2:1)
Responsible for ~5-20% of sudden cardiac death in young adults
COVID-19-associated myocarditis gained significant attention during the pandemic
PET-CT: may show active inflammation (useful in cardiac sarcoidosis)
Management
Non-pharmacological
Exercise restriction: avoid strenuous activity for ≥3-6 months (risk of arrhythmia and sudden death)
Return to exercise only after: symptoms resolved, LV function normalised, no significant arrhythmias, inflammatory markers normalised, MRI shows resolution
Bed rest in acute phase if symptomatic
Pharmacological
Heart failure management: ACEi/ARB, beta-blocker, diuretics, MRA - as per standard heart failure guidelines
Avoid NSAIDs: may worsen myocardial inflammation and impair healing (animal data)
Anticoagulation: consider if severe LV dysfunction or intracardiac thrombus
Antiarrhythmics: for symptomatic arrhythmias; avoid amiodarone long-term if possible
Immunosuppression (corticosteroids ± azathioprine): for specific subtypes:
NOT recommended for routine viral myocarditis (no proven benefit)
Surgical/Interventional
Mechanical circulatory support: IABP, Impella, ECMO for fulminant myocarditis with cardiogenic shock
Heart transplantation: for refractory end-stage heart failure
ICD: if persistent severe LV dysfunction (EF ≤35%) after ≥3-6 months of optimal medical therapy
Wearable defibrillator (LifeVest): may be used as bridge during recovery period
Referral Criteria
All suspected myocarditis: cardiology referral for cardiac MRI and management
Haemodynamic compromise: CCU/ICU admission, MCS team involvement
Suspected giant cell myocarditis: urgent EMB and immunosuppression
Prognosis
Mild/moderate viral myocarditis: ~50-70% recover fully with normalised LV function
~20-30% develop chronic dilated cardiomyopathy
Fulminant myocarditis: paradoxically, those who survive the acute phase (with intensive support) have better long-term outcomes (~90% survival) - vigorous immune response may clear virus effectively
Giant cell myocarditis: rapidly fatal without immunosuppression; median survival ~6 months untreated; transplant-free survival ~50% at 5 years with treatment
Sudden cardiac death: ~5-20% of unexplained SCD in young adults attributed to myocarditis
COVID-19 myocarditis: generally mild; severe cases rare but occurred
Other Relevant Information
Lake Louise Criteria for CMR Diagnosis of Myocarditis
Criterion
MRI Finding
Myocardial oedema
T2 hyperintensity or elevated T2 mapping
Hyperaemia/capillary leak
Early gadolinium enhancement
Necrosis/fibrosis
Late gadolinium enhancement (non-ischaemic pattern)
Left ventricular dilatation and systolic dysfunction not explained by coronary artery disease or abnormal loading conditions. The most common cardiomyopathy and a leading indication for heart transplantation.
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Most common cardiomyopathy: prevalence ~1 in 250-500; most common indication for heart transplantation
Idiopathic in ~50%; genetic/familial in ~25-30% (autosomal dominant; TTN gene most common mutation)
ICD if LVEF ≤35% despite ≥3 months optimal medical therapy (sudden death prevention)
CRT if LVEF ≤35% + LBBB QRS ≥150 ms + NYHA II-IV symptoms
Peripartum cardiomyopathy: occurs in last month of pregnancy or within 5 months postpartum; ~50% recover LV function
Genetic counselling: first-degree relatives should be screened with echo + ECG
Overview
Key Facts
Dilated cardiomyopathy (DCM) is characterised by left ventricular (or biventricular) dilatation with impaired systolic function, in the absence of coronary artery disease or abnormal loading conditions sufficient to explain the degree of dysfunction. It is the most common form of cardiomyopathy.
Epidemiology
Prevalence: ~1 in 250-500
Most common cardiomyopathy; accounts for ~50% of cardiomyopathies
BNP/NT-proBNP: elevated (severity and prognosis marker)
FBC, U&Es, LFTs: baseline
TFTs: thyroid dysfunction
Iron studies, ferritin: haemochromatosis
Glucose/HbA1c: diabetes
Vitamin B1 (thiamine): if nutritional deficiency suspected
Viral serology: if recent myocarditis suspected
Autoimmune screen: if connective tissue disease features
Imaging
Echocardiography: LV dilatation, reduced LVEF, global hypokinesis, functional MR/TR, LA dilatation
Cardiac MRI: gold standard for volumes and function; late gadolinium enhancement pattern (mid-wall fibrosis in DCM); excludes ischaemic cause, myocarditis, sarcoidosis
Coronary angiography: essential to exclude ischaemic aetiology
Special Tests
Genetic testing: recommended for all DCM patients (especially familial, young onset, conduction disease)
Family screening: ECG + echocardiography for first-degree relatives (repeat every 2-5 years)
Endomyocardial biopsy: rarely needed; consider for suspected giant cell myocarditis, sarcoidosis, or haemochromatosis
Holter monitor: arrhythmia assessment
Management
Non-pharmacological
Sodium restriction (<6g/day), fluid restriction (1.5-2L/day if hyponatraemic)
Inherited cardiac condition with asymmetric LV hypertrophy (≥15 mm) not explained by loading conditions. The most common cause of sudden cardiac death in young athletes.
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Most common inherited cardiac disease: prevalence ~1 in 500; autosomal dominant with variable penetrance
Sarcomeric gene mutations: MYH7 (beta-myosin heavy chain) and MYBPC3 (myosin-binding protein C) account for ~70% of genetic cases
Most common cause of sudden cardiac death in young athletes (<35 years)
LVOTO (left ventricular outflow tract obstruction): present in ~70%; worsened by Valsalva, standing, exercise, dehydration; improved by squatting
Murmur: harsh ejection systolic at LLSE, increases with Valsalva and standing (reduced preload increases obstruction)
Risk stratification for SCD: HCM Risk-SCD calculator (ESC); ICD for estimated 5-year risk ≥6%
Treatment: beta-blockers or verapamil first-line for symptoms; mavacamten (cardiac myosin inhibitor) is novel therapy for obstructive HCM
Avoid: vasodilators, high-dose diuretics, digoxin, and strenuous competitive exercise
Overview
Key Facts
Hypertrophic cardiomyopathy (HCM) is characterised by unexplained left ventricular hypertrophy (wall thickness ≥15 mm, or ≥13 mm with family history/positive genotype) not explained by abnormal loading conditions. It is the most common inherited cardiac disease.
Epidemiology
Prevalence: ~1 in 500 (0.2%); one of the most common inherited heart conditions
Equal sex distribution genotypically, but male predominance in clinical phenotype
Most commonly diagnosed in adolescence/young adulthood
Leading cause of SCD in young athletes (<35 years)
Aetiology
Autosomal dominant with variable penetrance and expressivity
Rare cardiomyopathy characterised by impaired ventricular filling due to increased myocardial stiffness with normal or near-normal wall thickness and systolic function. Most commonly caused by amyloidosis.
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Key Facts
Least common cardiomyopathy; characterised by diastolic dysfunction with preserved systolic function (until late)
Most common cause: cardiac amyloidosis (AL amyloidosis and ATTR amyloidosis)
Key differential: constrictive pericarditis - crucial to distinguish as CP is surgically curable
BNP markedly elevated (helps differentiate from constrictive pericarditis where BNP is mildly elevated)
Restrictive cardiomyopathy (RCM) is characterised by impaired ventricular filling due to increased stiffness of the myocardium, with normal or near-normal systolic function and wall thickness. It is the least common form of cardiomyopathy.
Epidemiology
Rarest form of cardiomyopathy (<5% of all cardiomyopathies)
Amyloidosis is the most common infiltrative cause
ATTR amyloidosis: increasingly recognised, especially in elderly (wild-type ATTR in ~13% of elderly patients with HFpEF)
Endomyocardial fibrosis: most common cause in tropical countries
Aetiology
Infiltrative:
Amyloidosis: AL (light-chain), ATTR (transthyretin - hereditary or wild-type)
Transient LV systolic dysfunction with apical ballooning, typically triggered by emotional or physical stress, mimicking acute MI but with unobstructed coronary arteries. Also known as stress cardiomyopathy.
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Also known as: stress cardiomyopathy, broken heart syndrome, apical ballooning syndrome
Predominantly affects postmenopausal women (~90% of cases; mean age 65-70)
Triggered by intense emotional or physical stress: bereavement, surgery, critical illness, natural disasters
Mimics ACS: chest pain, ST elevation (often anterior), troponin rise - but normal coronary arteries on angiography
Apical ballooning on echo/ventriculography with hyperkinetic base - resembles a Japanese octopus trap (takotsubo)
Usually reversible: LV function recovers within 4-8 weeks in most cases
Takotsubo cardiomyopathy (TTC) is an acute, usually reversible cardiomyopathy characterised by transient regional LV wall motion abnormalities (typically apical ballooning), occurring in the setting of emotional or physical stress, in the absence of obstructive coronary artery disease.
Epidemiology
Accounts for ~1-3% of all patients presenting with suspected ACS
~90% are postmenopausal women (age 60-75)
Rare in men; when it occurs, may have worse outcomes
Prevalence increasing (likely due to improved recognition)
Cardiac MRI: oedema without significant fibrosis; absence of LGE (differentiates from MI and myocarditis); wall motion abnormality extending beyond single coronary territory
InterTAK Diagnostic Criteria
Transient LV wall motion abnormality beyond single coronary territory
Emotional or physical trigger often (but not always) present
New ECG abnormalities, modest troponin rise
No obstructive coronary disease
No myocarditis (confirmed by MRI)
Postmenopausal women predominantly affected
Management
Non-pharmacological
Supportive care, cardiac monitoring
Psychological support and stress management
Avoid catecholamine-augmenting agents if possible
Pharmacological
Heart failure management: ACEi/ARB, beta-blockers, diuretics as needed
Anticoagulation: if LV thrombus present or LVEF severely reduced (warfarin or DOAC)
If LVOT obstruction: avoid inotropes and vasodilators
Phenylephrine (alpha-agonist) for hypotension with LVOTO
IV fluids
Short-acting beta-blocker (esmolol)
If no LVOTO with cardiogenic shock: cautious use of inotropes and IABP
Beta-blockers: may reduce recurrence (evidence limited)
Avoid dobutamine, adrenaline in LVOTO variant
Surgical/Interventional
Mechanical circulatory support (IABP, Impella, ECMO): for refractory cardiogenic shock without LVOTO
IABP contraindicated if significant LVOTO
Rarely needed; most recover with supportive care
Referral Criteria
All suspected Takotsubo: admission and coronary angiography to exclude ACS
Cardiogenic shock: CCU/ICU with advanced HF team involvement
Follow-up echocardiography at 4-8 weeks to confirm recovery
Prognosis
LV function recovers in ~95% of cases within 4-8 weeks
In-hospital mortality: ~4-5% (comparable to NSTEMI)
Cardiogenic shock: occurs in ~10%; mortality in this subgroup ~15-20%
Recurrence: ~5-10% over 5 years
Male patients: worse in-hospital outcomes
Long-term mortality after Takotsubo may be slightly higher than age-matched controls (ongoing research)
Most patients: excellent long-term prognosis after recovery
Inherited cardiomyopathy characterised by fibro-fatty replacement of the right ventricular myocardium, predisposing to ventricular arrhythmias and sudden cardiac death, particularly during exercise.
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Key Facts
Autosomal dominant with incomplete penetrance; desmosomal gene mutations (PKP2 most common) in ~60%
Fibro-fatty replacement of RV myocardium
arrhythmogenic substrate
VT/VF
Second most common cause of SCD in young athletes (after HCM)
ECG: T-wave inversions V1-V3, epsilon waves (small deflection at end of QRS in V1-V3), prolonged S-wave upstroke
2010 Task Force Criteria: combination of structural, histological, ECG, arrhythmia, and family history criteria
Exercise restriction: avoid competitive sport and endurance exercise (worsens disease progression)
ICD: for cardiac arrest survivors, sustained VT, or high-risk features
Overview
Key Facts
Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy characterised by progressive fibro-fatty replacement of the right ventricular myocardium, creating an arrhythmogenic substrate that predisposes to ventricular tachycardia, VF, and sudden cardiac death, particularly during exercise.
Epidemiology
Prevalence: ~1 in 2,000-5,000
More common in males (clinical expression) ~3:1
Mean age at presentation: 20-40 years
Endemic in certain regions: Veneto region of Italy, Naxos disease (Greece)
Second most common cause of SCD in young athletes after HCM
Congenital defect in the interatrial septum allowing left-to-right shunting. Secundum ASD is the most common type. Often presents in adulthood with dyspnoea, AF, or paradoxical embolism.
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Most common congenital heart defect diagnosed in adulthood; accounts for ~10% of all CHD
Secundum ASD (fossa ovalis) is the most common type (~70%); ostium primum associated with Down syndrome and AV canal defects
Auscultation: fixed widely split S2 (pathognomonic) + ejection systolic flow murmur at LUSE (increased flow through PV)
Left-to-right shunt
RV volume overload
pulmonary hypertension (decades)
Eisenmenger syndrome (reversal to right-to-left shunt)
Transcatheter device closure (Amplatzer): standard treatment for secundum ASD with significant shunt (Qp:Qs >1.5:1)
Paradoxical embolism: venous thromboembolism crossing to systemic circulation via ASD → stroke
NICE recommends closure for haemodynamically significant ASDs (Qp:Qs >1.5:1) with RV volume overload
Overview
Key Facts
Atrial septal defect (ASD) is a congenital defect in the interatrial septum allowing communication between the left and right atria. It is the most common congenital heart defect presenting in adulthood.
Epidemiology
~10% of all congenital heart defects; most common CHD diagnosed in adulthood
Female:male ratio ~2:1
Prevalence: ~1-2 per 1,000 live births
Many small ASDs close spontaneously in childhood
Types
Secundum (~70%): defect in fossa ovalis region; most common; amenable to device closure
Primum (~15-20%): defect in inferior septum near AV valves; associated with Down syndrome, AV canal defects, cleft mitral valve
Sinus venosus (~5-10%): defect near SVC or IVC entry; often associated with anomalous pulmonary venous drainage
Coronary sinus (<1%): unroofed coronary sinus
Pathophysiology
Left atrial pressure > right atrial pressure → left-to-right shunt
Chronic volume overload of RA and RV → RV dilatation, pulmonary overcirculation
Congenital defect in the interventricular septum allowing left-to-right shunting. The most common congenital heart defect overall. Small VSDs often close spontaneously; large VSDs require surgical repair.
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Key Facts
Most common congenital heart defect overall: ~25-30% of all CHD; incidence ~2-5 per 1,000 live births
Perimembranous (70-80%) is the most common type; muscular VSDs more likely to close spontaneously
Murmur: harsh pansystolic murmur at left lower sternal edge with thrill; louder murmur = smaller defect (restrictive VSD)
Small (restrictive) VSD: often close spontaneously by age 10 (~60-70%); loud murmur but minimal haemodynamic effect
Large (non-restrictive) VSD
significant left-to-right shunt
LV volume overload
heart failure
pulmonary hypertension
Eisenmenger if uncorrected
Surgical closure: indicated for large VSDs with significant shunt (Qp:Qs >2:1), heart failure, or failure to thrive
Ventricular septal defect (VSD) is a congenital defect in the interventricular septum allowing communication between the left and right ventricles. It is the most common congenital heart defect.
Epidemiology
Most common CHD: ~25-30% of all congenital heart defects
Incidence: ~2-5 per 1,000 live births
Equal sex distribution
~60-70% of small muscular VSDs close spontaneously by age 10
Types
Perimembranous (membranous) (~70-80%): adjacent to aortic and tricuspid valves; most common type
Muscular (~15-20%): within the muscular septum; multiple ('Swiss cheese') or single; highest spontaneous closure rate
Outlet (supracristal/subpulmonary) (~5-8%): near the pulmonary valve; higher in Asian populations; risk of aortic valve prolapse
Inlet (~5%): posterior septum near AV valves; associated with AVSD and Down syndrome
Pathophysiology
LV pressure > RV pressure → left-to-right shunt through VSD
Small (restrictive) VSD: high velocity jet through small defect; minimal haemodynamic effect; loud murmur (Maladie de Roger)
Large (non-restrictive) VSD
significant shunt
pulmonary overcirculation
LV volume overload
heart failure
Chronic pulmonary overcirculation
pulmonary vascular remodelling
pulmonary arterial hypertension
Eisenmenger syndrome
irreversible pulmonary hypertension
shunt reversal
cyanosis
Clinical Presentation
Small VSD
Usually asymptomatic
Loud pansystolic murmur heard incidentally
Thrill at left sternal edge
Normal growth and development
Large VSD (infants)
Heart failure symptoms: poor feeding, failure to thrive, sweating with feeds, tachypnoea
Recurrent chest infections
Hepatomegaly
Failure to gain weight
Examination Findings
Harsh pansystolic murmur at left lower sternal edge with thrill
Persistence of the fetal communication between the aorta and pulmonary artery after birth. Causes continuous left-to-right shunting with a characteristic continuous 'machinery' murmur.
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Key Facts
Ductus arteriosus normally closes within 24-48 hours of birth; failure to close = PDA
Continuous 'machinery' murmur best heard in the left infraclavicular region, loudest in late systole and enveloping S2
Risk factors: prematurity (most important), maternal rubella, high altitude, Down syndrome, fetal alcohol syndrome
Left-to-right shunt
pulmonary overcirculation
LV volume overload
heart failure if large; Eisenmenger if untreated
Medical closure: indomethacin or ibuprofen IV (inhibit prostaglandin synthesis) - effective in premature neonates
Surgical/transcatheter closure: indicated for haemodynamically significant PDA; transcatheter coil/device closure is standard in older infants and children
Bounding (collapsing) pulse and wide pulse pressure from aortic runoff into pulmonary artery
Prostaglandin E1 (alprostadil) infusion: keeps ductus OPEN in duct-dependent congenital heart lesions (e.g., critical coarctation, TGA)
Overview
Key Facts
Patent ductus arteriosus (PDA) is the persistence of the fetal vascular connection between the descending aorta and the pulmonary artery (main PA or left PA) after birth. It normally closes functionally within 24-48 hours.
Epidemiology
Accounts for ~5-10% of congenital heart defects
Incidence: ~1 in 2,000 term births; much higher in premature infants (~30-40% of infants <1,500g)
Female:male ratio ~2:1
More common at high altitude
Aetiology
Prematurity: most important risk factor (immature ductal wall lacks response to oxygen)
Maternal rubella infection (first trimester)
Genetic: Down syndrome, DiGeorge syndrome
Fetal alcohol syndrome
High altitude (lower PaO₂ fails to trigger ductal closure)
Pathophysiology
In fetal life, ductus arteriosus diverts blood from PA to aorta (bypassing lungs)
After birth, rising PaO₂ and falling prostaglandin levels trigger ductal constriction and closure
If ductus remains patent: aortic pressure > PA pressure → left-to-right shunt
Magnitude depends on duct size and pulmonary vascular resistance
Large PDA
pulmonary overcirculation
LV volume overload
heart failure
Long-term
pulmonary hypertension
Eisenmenger syndrome (shunt reversal
differential cyanosis: lower body cyanotic, upper body pink)
Clinical Presentation
Typical Presentation
Small PDA: asymptomatic; murmur found incidentally
Large PDA in infants: poor feeding, failure to thrive, tachypnoea, recurrent chest infections, heart failure
Adults with unrepaired PDA: dyspnoea, palpitations (AF), heart failure
Examination Findings
Continuous 'machinery' murmur: loudest at left infraclavicular area; peaks in late systole, continues through S2 into diastole
Bounding (collapsing) pulse: aortic runoff through ductus
Wide pulse pressure
Hyperdynamic apex (LV volume overload)
Thrill in left infraclavicular region
In Eisenmenger PDA: differential cyanosis (lower limbs cyanosed, upper limbs pink) and differential clubbing (toes clubbed, fingers not)
Red Flags
Heart failure in premature neonate
Differential cyanosis (Eisenmenger)
Failure to thrive
Wide pulse pressure with bounding pulses in an infant
Differential Diagnosis
Diagnosis
Key Features
Investigation
Venous hum
Continuous murmur in neck, abolished by lying down or compressing jugular
Clinical
AV fistula (coronary, pulmonary)
Continuous murmur, localisation varies
Echocardiography, CT angiography
Aortopulmonary window
Large communication between aorta and PA, often without continuous murmur
Congenital narrowing of the aorta, typically at the isthmus just distal to the left subclavian artery near the ligamentum arteriosum. Causes upper limb hypertension with reduced lower limb pulses.
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Key Facts
Location: typically at the aortic isthmus, distal to left subclavian artery near insertion of ligamentum arteriosum
Radio-femoral delay and upper limb hypertension with lower BP in legs - classic clinical finding
Four Ds: differential BP (arms > legs), delayed femoral pulses, dilated collaterals (intercostal), and diminished femoral pulses
Coarctation of the aorta is a congenital narrowing of the aorta, most commonly at the isthmus just distal to the origin of the left subclavian artery, near the insertion of the ligamentum arteriosum (ductus arteriosus remnant).
Epidemiology
Accounts for ~5-8% of congenital heart defects
Male:female ratio ~2:1 (except in Turner syndrome)
Incidence: ~3-4 per 10,000 live births
Associated with Turner syndrome (45,X) - coarctation occurs in ~10-15% of Turner patients
Aetiology
Congenital: abnormal development of the aortic arch
Two theories: (1) ectopic ductal tissue in aortic wall contracts after birth; (2) reduced aortic flow during fetal life (flow theory)
Strong association with bicuspid aortic valve (~50-80% of coarctation patients have BAV)
Most common cyanotic congenital heart disease comprising four defects: VSD, overriding aorta, RVOT obstruction, and RV hypertrophy. Presents with cyanosis and 'tet spells' in infancy.
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Key Facts
Four components: (1) large VSD, (2) overriding aorta, (3) RVOT obstruction (infundibular ± valvular PS), (4) RV hypertrophy
Most common cyanotic CHD: accounts for ~10% of all congenital heart defects
Boot-shaped heart on CXR (coeur en sabot) with oligaemic lung fields and right-sided aortic arch (~25%)
Complete surgical repair: definitive treatment, usually performed at age 3-6 months; involves VSD closure + RVOT reconstruction
Associated with: DiGeorge syndrome (22q11 deletion), Down syndrome; right-sided aortic arch in ~25%
Eisenmenger physiology from birth: unlike ASD/VSD, the right-to-left shunt is due to RVOT obstruction, NOT elevated PVR
Long-term complications post-repair: pulmonary regurgitation (most common), RV dilatation, arrhythmias (VT, AF), need for PVR
Overview
Key Facts
Tetralogy of Fallot (ToF) is the most common cyanotic congenital heart defect, comprising four anatomical abnormalities that result from anterior malalignment of the infundibular septum.
Epidemiology
Most common cyanotic CHD: ~10% of all congenital heart defects
Incidence: ~3-5 per 10,000 live births
Equal sex distribution
Aetiology
Anterior malalignment of the infundibular (conal) septum - this single embryological defect produces all four features
Genetic: 22q11.2 deletion (DiGeorge syndrome) in ~15%; Down syndrome; JAG1 mutations
VSD patch closure + RVOT reconstruction (resection of muscle bundles ± transannular patch if needed)
Timing: electively at 3-6 months of age
Operative mortality: <2% in experienced centres
Modified Blalock-Taussig (BT) shunt: palliative; systemic-to-PA shunt (subclavian to PA via Gore-Tex tube) for severely cyanotic neonates as bridge to definitive repair
Balloon pulmonary valvuloplasty: palliative if predominant valvular stenosis
Late pulmonary valve replacement (PVR): for severe PR following repair (timing guided by RV volumes on MRI)
Referral Criteria
All cyanotic neonates: immediate neonatal/paediatric cardiology referral
Life-threatening emergency caused by a tear in the aortic intima allowing blood to enter the media, creating a false lumen. Type A involves ascending aorta requiring emergency surgery; Type B involves descending aorta managed medically.
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Key Facts
Stanford classification: Type A = ascending aorta (requires emergency surgery); Type B = descending aorta only (medical management unless complicated)
Tearing/ripping chest or interscapular back pain of sudden onset - classically migratory as dissection propagates
BP differential >20 mmHg between arms is suggestive but not always present
Overview
Key Facts
Aortic dissection is a cardiovascular emergency in which a tear in the aortic intima allows blood to enter the media, creating a false lumen that can propagate proximally or distally, compromising branch vessel perfusion and risking aortic rupture.
Epidemiology
Incidence: ~3-5 per 100,000 per year
Male:female ratio ~3:1
Peak incidence: 60-70 years (degenerative); younger in connective tissue disorders
Type A: ~65% of dissections; Type B: ~35%
Aetiology
Hypertension: most important risk factor (~70-80% of patients)
Connective tissue disorders: Marfan syndrome (FBN1), Ehlers-Danlos type IV (COL3A1), Loeys-Dietz
Bicuspid aortic valve: associated aortopathy
Aortic aneurysm: pre-existing dilatation
Cocaine use: acute hypertensive surge
Turner syndrome, coarctation of the aorta
Pregnancy: typically 3rd trimester or peripartum
Iatrogenic: post-cardiac surgery, catheterisation
Trauma: deceleration injuries
Pathophysiology
Intimal tear allows high-pressure blood to enter the media → dissection plane propagates
False lumen may compress true lumen → branch vessel malperfusion
Propagation can extend proximally (into aortic root → AR, coronary occlusion, tamponade) or distally (abdominal aorta, iliac arteries)
Rupture may occur into pericardium (tamponade), pleural space, or mediastinum
Clinical Presentation
Typical Presentation
Sudden-onset severe chest or interscapular back pain: described as 'tearing' or 'ripping'
Pain may migrate as dissection propagates
Type A: anterior chest pain
Type B: interscapular back pain
Associated: diaphoresis, nausea, syncope
Examination Findings
Blood pressure differential >20 mmHg between arms
Pulse deficit (absent or reduced pulse in one limb)
Aortic regurgitation murmur (type A involving aortic root)
Hypotension/shock (tamponade, rupture, severe AR)
Unilateral weakness: stroke from carotid malperfusion
Permanent dilatation of the abdominal aorta to ≥3 cm, most commonly infrarenal. Usually asymptomatic until rupture, which carries ~80% overall mortality. UK NHS screening programme for men at age 65.
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Key Facts
Definition: aortic diameter ≥3 cm (normal infrarenal aorta ~2 cm); most commonly infrarenal (95%)
Risk factors: smoking (strongest modifiable), male sex, age >65, family history, hypertension, atherosclerosis
Usually asymptomatic: detected incidentally or through NHS AAA screening programme
NHS AAA screening: ultrasound offered to all men at age 65 in England
Elective repair: indicated when diameter ≥5.5 cm (men) or ≥5.0 cm (women), or growth ≥1 cm/year, or symptomatic
EVAR (endovascular repair) vs open surgical repair: EVAR has lower perioperative mortality (~1-2% vs ~5%) but higher re-intervention rate
Ruptured AAA: triad of sudden abdominal/back pain, hypotension, pulsatile abdominal mass - requires emergency surgery; overall mortality ~80%
UK Aneurysm Growth Study and EVAR-1/EVAR-2 trials: guided current thresholds and repair strategies
Overview
Key Facts
Abdominal aortic aneurysm (AAA) is a permanent dilatation of the abdominal aorta to a diameter of ≥3 cm. It most commonly affects the infrarenal aorta. The major risk is rupture, which is often fatal.
Epidemiology
Prevalence: ~4-8% of men >65 years; ~1-2% of women >65 years
Male:female ratio ~6:1
UK NHS screening has reduced AAA-related mortality
Dilatation of the thoracic aorta to ≥150% of normal diameter. Often asymptomatic, with risk of rupture and dissection. Frequently associated with connective tissue disorders and bicuspid aortic valve.
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Key Facts
Defined as thoracic aortic diameter ≥50% greater than normal (ascending aorta normal ~3.5 cm)
Usually asymptomatic: found incidentally on imaging; may cause chest/back pain, hoarseness, dysphagia
Rupture and dissection are the major complications; risk increases with size
Surgical threshold: ≥5.5 cm for ascending; ≥6.0 cm for descending; lower thresholds for Marfan (≥4.5-5.0 cm) and BAV (≥5.0 cm)
Open surgical repair (ascending) or TEVAR (descending) are standard approaches
CT angiography or MR angiography: for diagnosis, monitoring, and surgical planning
First-degree relative screening: recommended if familial TAA or connective tissue disorder
Overview
Key Facts
Thoracic aortic aneurysm (TAA) is abnormal dilatation of the thoracic aorta. It may involve the ascending aorta, aortic arch, or descending thoracic aorta. TAA may be asymptomatic for years but carries significant risk of rupture and dissection.
Epidemiology
Incidence: ~6-10 per 100,000 per year
Male:female ratio ~2-3:1
Ascending aorta aneurysms account for ~60%
Associated with connective tissue disorders in ~20% of cases
Aetiology
Degenerative (medial degeneration): most common; associated with ageing, hypertension, atherosclerosis
Connective tissue disorders: Marfan (FBN1), Loeys-Dietz (TGFBR1/2), Ehlers-Danlos type IV (COL3A1)
Bicuspid aortic valve: BAV-associated aortopathy, even without significant valve disease
Atherosclerotic narrowing of the peripheral arteries, most commonly affecting the lower limbs, causing intermittent claudication and, in severe cases, critical limb ischaemia with rest pain and tissue loss.
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Key Facts
Intermittent claudication: cramping leg pain on walking, relieved by rest - most common presentation
Ankle-brachial pressure index (ABPI): key diagnostic tool; ABPI <0.9 diagnostic of PAD; <0.5 suggests critical ischaemia
Risk factors: smoking (strongest), diabetes, hypertension, hyperlipidaemia, age >50
Fontaine classification
Stage I asymptomatic
II claudication
III rest pain
IV tissue loss (ulceration/gangrene)
NICE CG147: supervised exercise programme (SEP) for ≥3 months is first-line for intermittent claudication
Medical therapy: aspirin or clopidogrel, statin, antihypertensive, smoking cessation
Revascularisation: angioplasty ± stenting or bypass surgery for critical limb ischaemia or lifestyle-limiting claudication despite exercise therapy
PAD is a marker of systemic atherosclerosis: patients have ~3-fold increased risk of MI and stroke
Overview
Key Facts
Peripheral arterial disease (PAD) is the manifestation of atherosclerosis in the arteries supplying the lower limbs. It ranges from asymptomatic disease to intermittent claudication and critical limb ischaemia.
Epidemiology
Affects ~13% of people >50 years; prevalence increases with age
Only ~25% of PAD patients are symptomatic
Male:female ratio ~2:1
UK prevalence: ~20% of people >60 years have some evidence of PAD on ABPI
Aetiology
Atherosclerosis - identical process to coronary and cerebral artery disease
Risk factors: smoking (OR ~3-5), diabetes, hypertension, hyperlipidaemia, CKD, family history, age, male sex
Non-atherosclerotic: Buerger disease (thromboangiitis obliterans - young male smokers), popliteal entrapment, fibromuscular dysplasia
Pathophysiology
Atherosclerotic plaque formation → progressive stenosis of peripheral arteries
Sudden decrease in limb perfusion threatening tissue viability, requiring emergency revascularisation within 6 hours to prevent irreversible damage and limb loss. Causes include embolism and thrombosis in situ.
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Key Facts
6 Ps: Pain, Pallor, Pulselessness, Paraesthesia, Paralysis, Perishingly cold - Paralysis and paraesthesia indicate irreversible damage is imminent
Time-critical emergency: revascularisation needed within 4-6 hours to prevent irreversible ischaemia
Embolism (most common cause): usually cardiac origin - AF, MI with mural thrombus, prosthetic valve; sudden onset, no claudication history
Thrombosis in situ: on pre-existing atherosclerotic disease; more gradual onset, history of claudication, collateral circulation
CT angiography: urgent imaging to plan intervention
Treatment: emergency surgical embolectomy (Fogarty catheter) or catheter-directed thrombolysis (alteplase); systemic IV heparin immediately
Compartment syndrome: risk after reperfusion (reperfusion injury); monitor for tense swollen leg, severe pain, paraesthesia - requires emergency fasciotomy
Rutherford classification grades severity from viable (I) to irreversible (III)
Overview
Key Facts
Acute limb ischaemia (ALI) is a sudden decrease in limb perfusion that threatens tissue viability. It is a vascular emergency requiring immediate assessment and intervention.
Epidemiology
Incidence: ~14 per 100,000 per year
More common in elderly; associated with cardiovascular disease
Embolism:thrombosis ratio ~4:1 in historical series; thrombosis now more common due to ageing population with PAD
Aetiology
Embolism (~40-50%):
Cardiac: AF (most common source), MI with mural thrombus, prosthetic valve, endocarditis, atrial myxoma
Blood clot formation in the deep venous system, most commonly in the lower limbs. Major risk is pulmonary embolism. Diagnosed with D-dimer and compression ultrasonography; treated with anticoagulation.
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Wells score for DVT: clinical probability assessment guiding investigation pathway
D-dimer: highly sensitive but low specificity; negative D-dimer with low Wells score effectively excludes DVT
Compression ultrasonography: diagnostic investigation of choice; non-compressible vein = DVT
First-line treatment: DOAC (rivaroxaban 15mg BD for 3 weeks then 20mg OD, or apixaban 10mg BD for 7 days then 5mg BD) per NICE NG158
Proximal DVT (above knee) carries higher PE risk than distal (below knee) DVT
Duration of anticoagulation: 3 months for provoked; 3-6 months or lifelong for unprovoked or recurrent
Post-thrombotic syndrome: chronic complication (~30-50%) with leg swelling, pain, skin changes, venous ulceration
Overview
Key Facts
Deep vein thrombosis (DVT) is the formation of a blood clot in the deep venous system, most commonly in the lower limbs. It is part of the spectrum of venous thromboembolism (VTE), which includes DVT and pulmonary embolism (PE).
Epidemiology
VTE incidence: ~1-2 per 1,000 per year
DVT accounts for ~2/3 of VTE events
Increases with age: ~1 per 10,000 in young adults; ~1 per 100 in elderly
Recurrence rate: ~5-10% per year after first unprovoked DVT
Tender, palpable, erythematous cord along superficial vein
Clinical, USS
Lymphoedema
Non-pitting oedema, skin thickening, chronic
Clinical, imaging
Heart failure
Bilateral oedema, dyspnoea, JVP elevation
Echo, BNP
Diagnosis / Investigation
Wells Score for DVT
Criterion
Points
Active cancer (treatment within 6 months)
+1
Paralysis, paresis, or recent plaster immobilisation of lower limb
+1
Recently bedridden >3 days or major surgery within 12 weeks
+1
Localised tenderness along deep venous system
+1
Entire leg swollen
+1
Calf swelling >3 cm compared to asymptomatic side
+1
Pitting oedema confined to symptomatic leg
+1
Collateral superficial veins (non-varicose)
+1
Previously documented DVT
+1
Alternative diagnosis at least as likely as DVT
-2
≤1
DVT unlikely
D-dimer
if negative, DVT excluded
≥2: DVT likely → proximal leg vein USS within 4 hours (or D-dimer + interim anticoagulation if USS delayed)
Bloods
D-dimer: highly sensitive (>95%); low specificity; useful to exclude DVT in low-probability patients
FBC, U&Es, LFTs, coagulation: baseline
Thrombophilia screen: if unprovoked DVT in young patient (defer testing until after anticoagulation - Factor V Leiden, prothrombin mutation, antithrombin, protein C/S, antiphospholipid antibodies)
Cancer screen: CT CAP if unprovoked DVT (NICE recommendation), PSA, mammography as appropriate
Imaging
Compression ultrasonography: first-line imaging; sensitivity ~95% for proximal DVT, lower for distal
Positive: vein non-compressible
Whole-leg ultrasound: detects both proximal and distal DVT
CT venography: if USS inconclusive
MR venography: pelvic/IVC thrombosis
Management
Non-pharmacological
Early mobilisation: encouraged (bed rest not necessary)
Graduated compression stockings: NOT routinely recommended for post-thrombotic syndrome prevention (SOX trial negative); may be used for symptom relief
Pharmacological
Anticoagulation (NICE NG158):
DOACs first-line:
Rivaroxaban: 15mg BD for 21 days, then 20mg OD
Apixaban: 10mg BD for 7 days, then 5mg BD
No need for initial heparin bridging
Alternative: LMWH (enoxaparin 1.5mg/kg OD or dalteparin 200 IU/kg OD) + warfarin (target INR 2.0-3.0); stop LMWH when INR >2 for ≥24 hours
LMWH monotherapy: preferred in active cancer (or DOAC - NICE now permits edoxaban or rivaroxaban for cancer-associated VTE)
Dilated, tortuous superficial veins of the lower limbs resulting from incompetent venous valves. Common and usually cosmetic, but may cause symptoms or progress to chronic venous insufficiency.
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Key Facts
Prevalence: ~25-30% of adults; more common in women, increasing with age, obesity, pregnancy, and prolonged standing
Pathophysiology
superficial venous valve incompetence
venous reflux
venous hypertension
vein dilatation
Great saphenous vein (GSV) is most commonly affected; saphenofemoral junction incompetence is the commonest cause
NICE CG168: refer for treatment if symptomatic (aching, heaviness, itching, skin changes) or complications (bleeding, superficial thrombophlebitis, lipodermatosclerosis, venous ulceration)
Treatment: endothermal ablation (radiofrequency/laser) is first-line per NICE; alternatives include foam sclerotherapy and surgery (stripping)
Compression stockings: NOT first-line treatment (NICE CG168); used for symptom management or while awaiting treatment
Overview
Key Facts
Varicose veins are dilated, tortuous superficial veins, most commonly affecting the lower limbs. They result from incompetent venous valves causing reflux and venous hypertension.
Epidemiology
Prevalence: ~25-30% of Western adults
Female > male (~2:1)
Increases with age, parity, obesity
Risk factors: family history, prolonged standing, obesity, pregnancy, DVT history
Transient loss of consciousness due to transient global cerebral hypoperfusion, characterised by rapid onset, short duration, and spontaneous complete recovery. Most commonly vasovagal (neurally mediated).
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Key Facts
Definition: transient loss of consciousness (TLOC) due to global cerebral hypoperfusion with rapid onset, short duration, and spontaneous recovery
Most common cause: vasovagal syncope (neurocardiogenic) - triggered by prolonged standing, pain, emotional stress
Cardiac syncope carries the highest risk: arrhythmias (VT, bradycardia, long QT), structural (AS, HCM, PE), and must be excluded
Red flags for cardiac syncope: exertional, palpitations preceding, family history of SCD, abnormal ECG, structural heart disease
Orthostatic hypotension: SBP drop ≥20 mmHg or DBP drop ≥10 mmHg within 3 minutes of standing
All patients need 12-lead ECG - look for long QT, Brugada, WPW, heart block, HCM, ARVC features
NICE TA95: tilt table testing for recurrent unexplained syncope; implantable loop recorder (ILR) for infrequent episodes
Treatment: depends on cause - reassurance and conservative for vasovagal; pacemaker for cardioinhibitory; ICD for ventricular arrhythmias
Overview
Key Facts
Syncope is a transient loss of consciousness (TLOC) caused by transient global cerebral hypoperfusion. It is common, affecting up to 40% of the population at some point, and ranges from benign (vasovagal) to life-threatening (cardiac arrhythmia).
Epidemiology
Lifetime prevalence: ~40% of the general population
Accounts for ~1-3% of ED attendances and ~1-6% of hospital admissions
Vasovagal syncope: most common cause (~50-60% of all syncope)
Cardiac syncope: ~10-20% of cases but carries highest mortality
Classification
1. Neurally mediated (reflex) syncope:
Vasovagal: most common; triggered by prolonged standing, pain, emotion, heat
Cessation of effective cardiac mechanical activity confirmed by absence of pulse and responsiveness. Managed according to Resuscitation Council UK ALS guidelines with CPR, defibrillation for shockable rhythms, and reversible cause correction.
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Key Facts
Out-of-hospital cardiac arrest (OHCA): ~30,000 cases per year in the UK; overall survival to hospital discharge ~8-10%
Chain of survival: (1) early recognition + call for help, (2) early CPR, (3) early defibrillation, (4) post-resuscitation care
Shockable rhythms (VF/pulseless VT): defibrillation is the definitive treatment; each minute of delay reduces survival by ~10%
Non-shockable rhythms (PEA/asystole): worse prognosis; focus on identifying and treating reversible causes (4 Hs and 4 Ts)
Adrenaline 1mg IV every 3-5 minutes during CPR; amiodarone 300mg IV after 3rd shock for refractory VF/pVT
Targeted temperature management (TTM): maintain temperature ≤37.5°C for at least 72 hours post-ROSC (TTM2 trial)
Post-ROSC care: 12-lead ECG, coronary angiography if STEMI, ICU admission, neuroprognostication at ≥72 hours
Overview
Key Facts
Cardiac arrest is the cessation of effective cardiac mechanical activity, resulting in loss of consciousness and absence of pulse. Survival depends on rapid initiation of the chain of survival.
Epidemiology
OHCA: ~30,000 per year in the UK; survival to discharge ~8-10%
In-hospital cardiac arrest (IHCA): ~23,000 per year in the UK; survival to discharge ~25%
Shockable rhythms: ~20-25% of OHCA (higher survival); ~20% of IHCA
Male:female ratio ~2:1
Aetiology
Cardiac (~80%):
Coronary artery disease / acute MI: most common cause
Haemodynamic support: IV fluids, vasopressors (noradrenaline) to maintain MAP ≥65 mmHg
Targeted temperature management: maintain temperature ≤37.5°C for ≥72 hours (TTM2 trial: no benefit of targeting 33°C over 36°C, but fever prevention is key)
Coronary angiography: immediately if STEMI; consider early angiography in other cardiac arrest survivors
ICU admission: neurological monitoring, organ support
Elevated blood lipid levels (cholesterol and/or triglycerides) representing a major modifiable cardiovascular risk factor. High-intensity statin therapy is the cornerstone of management per NICE CG181.
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Key Facts
NICE CG181: offer atorvastatin 20mg OD for primary prevention if 10-year QRISK3 ≥10%; 80mg OD for secondary prevention (established CVD)
LDL-C is the primary target: aim for >40% reduction from baseline (NICE); ESC targets LDL <1.4 mmol/L for very high-risk patients
Familial hypercholesterolaemia (FH): autosomal dominant; LDL receptor, ApoB, or PCSK9 mutations; total cholesterol often >7.5 mmol/L; premature CVD
Simon Broome criteria and Dutch Lipid Clinic Network criteria: used for FH diagnosis
Additional therapies: ezetimibe 10mg OD (add to statin if target not met), PCSK9 inhibitors (alirocumab, evolocumab - NICE TA394/TA393), inclisiran (siRNA - TA733)
Statin side effects: myalgia (~10%), rhabdomyolysis (rare), hepatotoxicity (monitor LFTs), new-onset diabetes (~1 per 200 over 5 years)
Non-HDL cholesterol: increasingly used as target; includes all atherogenic lipoproteins
Overview
Key Facts
Hyperlipidaemia refers to elevated levels of lipids in the blood, primarily total cholesterol, LDL cholesterol, and triglycerides. It is a major modifiable risk factor for atherosclerotic cardiovascular disease.
Epidemiology
~60% of UK adults have total cholesterol >5 mmol/L
~1 in 250 people have heterozygous FH (under-diagnosed)
Systematic evaluation of an individual's risk of developing atherosclerotic cardiovascular disease over 10 years. QRISK3 is the recommended tool in the UK, guiding decisions on statin therapy and lifestyle interventions.
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Key Facts
QRISK3 is the recommended risk calculator in the UK (NICE CG181): estimates 10-year risk of MI or stroke
QRISK3 ≥10%: offer atorvastatin 20mg OD for primary prevention after lifestyle discussion
Risk factors in QRISK3: age, sex, ethnicity, smoking, systolic BP, cholesterol ratio, BMI, diabetes, CKD, AF, rheumatoid arthritis, family history of premature CVD, deprivation score, and more
Not suitable for: patients with established CVD (already high risk - treat directly), FH, type 1 diabetes, CKD stage 3-5 (already high risk), age <25 or >84
Framingham score and SCORE/SCORE2 are used in other countries; QRISK3 is validated for UK population
Non-modifiable risk factors: age, sex, ethnicity, family history, genetic factors
NHS Health Check: offered to adults aged 40-74 in England every 5 years for CVD risk assessment
Overview
Key Facts
Cardiovascular risk assessment (CVRA) is the systematic evaluation of risk factors to estimate an individual's likelihood of developing atherosclerotic cardiovascular disease (MI, stroke, PAD) over a defined time period, typically 10 years.
Epidemiology
CVD is the second leading cause of death in the UK (~25% of all deaths)
~7.6 million people in the UK live with CVD
CVD is largely preventable through risk factor modification
NHS Health Check programme aims to identify and manage high-risk individuals
Risk Factors
Non-modifiable:
Age (risk increases with age)
Sex (men at higher risk; women catch up post-menopause)
Ethnicity (South Asian and Black African/Caribbean at higher risk)
Family history of premature CVD (first-degree relative: male <55, female <65)
Genetic factors
Modifiable:
Smoking
Hypertension
Dyslipidaemia (high LDL, low HDL)
Diabetes mellitus
Obesity (especially central)
Physical inactivity
Excess alcohol
Chronic stress, depression
Chronic kidney disease
Inflammatory conditions (RA, SLE)
Atrial fibrillation
Clinical Presentation
Indications for Risk Assessment
All adults aged 40-74 without pre-existing CVD (NHS Health Check)
Younger adults with significant risk factors
Patients with conditions that increase CVD risk (CKD, diabetes, RA, etc.)
Assessment Components
Age, sex, ethnicity
Smoking status
Blood pressure (clinic BP)
Total cholesterol and HDL cholesterol (non-fasting acceptable)
Body mass index
Medical history: diabetes, CKD, AF, RA, migraine, SLE, erectile dysfunction, severe mental illness, atypical antipsychotic use
Family history of premature CVD
Townsend deprivation score (postcode-based in QRISK3)
When NOT to Use QRISK3
Established CVD (already secondary prevention → treat directly)
Type 1 diabetes >40 years or with >10 years duration or nephropathy/other risk factors (treat as high risk)
Familial hypercholesterolaemia (treat directly)
CKD stage 3-5 (already at increased risk)
Age <25 or >84 years
Differential Diagnosis
Tool
Region
Notes
QRISK3
UK
Recommended by NICE; UK-validated
Framingham Risk Score
USA
Older; overestimates risk in UK population
SCORE/SCORE2
Europe (ESC)
European populations; recalibrated by country
ASCVD Pooled Cohort
USA (ACC/AHA)
US guidelines
Reynolds Risk Score
USA
Includes CRP and family history
Diagnosis / Investigation
Required Measurements
Blood pressure: clinic BP (ideally average of 2 readings)
Lipid profile: total cholesterol, HDL-C (non-fasting acceptable per NICE)
Structured programme of exercise, education, and psychological support for patients recovering from cardiac events or procedures. Reduces cardiovascular mortality and hospital readmissions.
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Key Facts
Reduces cardiovascular mortality by ~25% and hospital readmissions by ~18-25% (Cochrane meta-analysis)
NICE CG172: all patients should be offered cardiac rehabilitation after MI, CABG, PCI, heart failure, and stable angina
BACPR (British Association for Cardiovascular Prevention and Rehabilitation) sets standards for CR in the UK
Uptake remains poor: only ~50% of eligible patients in the UK participate; lower in women, elderly, ethnic minorities
Home-based CR: equivalent outcomes to centre-based (BRUM trial); improves access and uptake
Exercise prescription: individualised, typically moderate intensity aerobic (60-80% max HR) + resistance training, 2-3 sessions/week for 6-12 weeks
Overview
Key Facts
Cardiac rehabilitation (CR) is a comprehensive, evidence-based programme of exercise, education, risk factor modification, and psychological support designed to improve outcomes and quality of life after cardiac events or procedures.
Epidemiology
~350,000 eligible patients per year in the UK
Uptake: ~50% of eligible patients (improving but still suboptimal)
Lower participation among women (~40%), elderly, ethnic minorities, and socioeconomically deprived populations
NACR (National Audit of Cardiac Rehabilitation) monitors outcomes and quality
Indications (NICE CG172)
Post-MI (STEMI/NSTEMI)
Post-PCI
Post-CABG
Stable angina
Heart failure (HFrEF and HFpEF)
Post-cardiac surgery (valve replacement)
Post-ICD/CRT implantation
Post-cardiac transplant
Stable PAD
Clinical Presentation
Who Should Be Referred
All patients after acute coronary syndrome (STEMI, NSTEMI, UA)
All patients after coronary revascularisation (PCI, CABG)
Pharmacological prevention and treatment of thromboembolic disease using parenteral (heparin) and oral (warfarin, DOACs) agents. Underpins management of AF, VTE, and mechanical heart valves.
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Key Facts
DOACs (apixaban, rivaroxaban, edoxaban, dabigatran) are first-line for non-valvular AF and VTE per NICE NG196
Warfarin remains indicated for mechanical heart valves and antiphospholipid syndrome - target INR 2.0–3.0 (or 2.5–3.5 for mechanical mitral valves)
Apixaban 5mg BD preferred DOAC in AF (RE-LY, ROCKET-AF, ARISTOTLE, ENGAGE AF-TIMI 48 trials)
CHA₂DS₂-VASc score guides anticoagulation in AF: offer anticoagulation if score ≥2 (men) or ≥3 (women)
LMWH (e.g. enoxaparin 1.5mg/kg OD or 1mg/kg BD) is first-line parenteral anticoagulant for VTE treatment
HIT (heparin-induced thrombocytopenia): immune-mediated, typically day 5-14; switch to argatroban or fondaparinux
Reversal agents: vitamin K/PCC for warfarin; idarucizumab for dabigatran; andexanet alfa for factor Xa inhibitors
Bleeding risk: assess with HAS-BLED score but high score alone should not preclude anticoagulation
Overview
Key Facts
Anticoagulation therapy is fundamental to the prevention and treatment of arterial and venous thromboembolism. The choice of agent depends on the indication, patient characteristics, renal function, and the balance of thrombotic versus bleeding risk.
Epidemiology
Approximately 1.4 million people in the UK take anticoagulants
AF affects ~1.5 million people in the UK; anticoagulation reduces stroke risk by ~65%
VTE affects ~1 in 1,000 per year in the UK
DOAC prescribing has overtaken warfarin since ~2018
Aetiology
Anticoagulation is used in:
Atrial fibrillation: stroke prevention
Venous thromboembolism: treatment and secondary prevention of DVT/PE
Mechanical heart valves: lifelong warfarin
Antiphospholipid syndrome: warfarin preferred
Post-ACS: short-term in combination with antiplatelet therapy
Perioperative thromboprophylaxis: LMWH
Pathophysiology
Warfarin: inhibits vitamin K-dependent clotting factors (II, VII, IX, X) and proteins C and S
DOACs: directly inhibit thrombin (dabigatran) or factor Xa (apixaban, rivaroxaban, edoxaban)
Heparins: potentiate antithrombin III activity - UFH inhibits thrombin + Xa; LMWH primarily inhibits Xa
Fondaparinux: selective factor Xa inhibitor via antithrombin
Clinical Presentation
Indications for Anticoagulation
Atrial fibrillation with CHA₂DS₂-VASc ≥2 (men) or ≥3 (women)
VTE: acute DVT or PE, and secondary prevention
Mechanical heart valves: lifelong warfarin
Post-ACS: short-term anticoagulation
Thromboprophylaxis: post-surgical, immobility, medical inpatients