Valvular heart disease

• Valvular heart disease (VHD) means one or more of the four heart valves (aortic, mitral, tricuspid, pulmonary) is narrowed (stenosis) or leaky (regurgitation). The question is always the same: is the valve causing symptoms or damaging the heart?
• Each lesion is described in the same way here: cause, mechanism, symptoms and murmur, investigations, severity on echo, and when to intervene.
• There is no drug that reverses a narrowed valve. Hypertension control, heart-failure medicines and diuretics relieve symptoms; valve intervention (repair or replacement) is the definitive treatment for severe disease.
• Severity is graded mainly by echocardiography, and staging (A-D) tells you when to watch and when to act.
• Rheumatic heart disease (RHD) remains the commonest cause of VHD worldwide and is the reason mitral stenosis is a disease of young people in Africa and other low-resource settings.

By the end of this page you should be able to:

1. List the major valve lesions and the key distinguishing features of each murmur.
2. Grade severity of the common lesions on echocardiography and assign Stages A-D.
3. Choose between watchful waiting, medical therapy, and valve intervention (surgical valve replacement, TAVI, balloon valvotomy, TEER) for a given patient, including timing.
4. State the INR targets after mechanical and bioprosthetic valve surgery and the anticoagulation rules in pregnancy.
5. Describe the current limited indications for infective endocarditis (IE) prophylaxis and the benzathine penicillin regimens for secondary prevention of rheumatic fever.
6. Apply the follow-up imaging intervals and the guidance from the 2020 AHA/ACC and 2021 ESC/EACTS valve guidelines.

Each major lesion below may later be given its own topic; this page is the overview that keeps them in one frame.

Each lesion is treated separately below. One overarching rule: the same patient can have several valve lesions, and when more than one valve is involved the predominant lesion usually decides the plan. ^[5]

Degenerative (calcific) disease dominates in ageing, high-income populations; rheumatic disease dominates worldwide and in low- and middle-income countries. RHD affects about 40.5 million people, with about 152,700 new cases a year in ESC member countries alone. ^[5] In children aged 5 to 14 years the global prevalence is roughly 1 in 1000. ^[5]

• Aortic stenosis (AS) is the commonest valve lesion in high-income countries; degenerative in about 82% of cases, rheumatic in about 11%, congenital (usually bicuspid) in about 6%, and post-endocarditis in about 1%. ^[5] About 40% of people over 75 have aortic sclerosis, and 3.4% have severe stenosis. ^[5]
• Aortic regurgitation (AR) reaches roughly 1.6% of people over 65 in the United Kingdom and 4.9% in the Framingham cohort. ^[5]
• Mitral regurgitation (MR) is the second commonest lesion in Europe (causes: ischaemic 30%, dilated cardiomyopathy 26%, prolapse 22%, rheumatic 16%). ^[5] Mitral valve prolapse occurs in 2 to 4% of people. ^[5]
• Tricuspid regurgitation (TR) affects about 4% of people over 75; about 95% is secondary. ^[5]

• Degenerative disease: age, hypertension, hyperlipidaemia, diabetes, chronic kidney disease.
• Rheumatic: previous acute rheumatic fever after group A streptococcal pharyngitis; overcrowding and low socioeconomic status.
• Congenital: bicuspid aortic valve, mitral valve prolapse, pulmonary stenosis.
• Other: endocarditis, radiation, connective tissue disorders (Marfan, Turner), carcinoid, drugs.

A stiff or leaking valve imposes a pressure load (stenosis) or a volume load (regurgitation) on the chamber behind it. The heart compensates: concentric hypertrophy for pressure load, eccentric hypertrophy and dilatation for volume load. Symptoms appear when compensation fails - raised filling pressures in the lungs or right heart, and a fall in forward output.

Murmurs follow from the same mechanics and are summarised in cardiac physical examination: systolic ejection murmur in AS, early diastolic blowing murmur in AR, mid-diastolic rumble in MS, holosystolic murmur in MR and TR. An ECG (see ECG interpretation) shows hypertrophy, atrial enlargement or arrhythmia but does not diagnose valve disease. ^[1]

• Transthoracic echocardiography (TTE) is the first-line test: valve anatomy, severity of stenosis/regurgitation, chamber size and function, and pulmonary pressures. ^[5]
• Transoesophageal echocardiography (TEE) clarifies mitral anatomy, flail segments, prosthetic dysfunction, and guides surgery, TAVI and TEER.
• Cardiac CT is used for calcium scoring (discordant AS), aortic annulus sizing before TAVI, and paravalvular leak assessment.
• Cardiac MRI helps when echo is inadequate or quantitative regurgitant volumes are needed.
• Exercise testing (symptom-limited, with blood pressure response) is useful in asymptomatic severe AS. ^[1][7]
• Coronary angiography or CT coronary angiography is required before valve surgery in older adults or with risk factors. ^[1]

Severity thresholds on echo are given with each lesion below. Where the two guidelines differ, both values are given.

The 2020 AHA/ACC system classifies every valve lesion by anatomy, severity, ventricular response and symptoms. ^[1]

Cause. Degenerative calcification in older adults; bicuspid valve in the young; rheumatic in low-resource settings. ^[5]

Pathophysiology. Pressure overload produces concentric left ventricular (LV) hypertrophy; the valve area narrows, gradients rise. Symptoms (angina, syncope, exertional breathlessness) appear late, when the valve is severely stenotic or the ventricle fails.

Symptoms/signs. Exertional dyspnoea, angina, syncope, a delayed and small-volume pulse, a sustained (heaving) apex, and a systolic ejection murmur radiating to the carotids. An ejection click suggests a bicuspid valve.

Investigations/grading (echo). Moderate AS has peak velocity 3.0-3.9 m/s, mean gradient 20-39 mm Hg, valve area 1.0-1.4 cm2. Severe AS: peak velocity ≥4.0 m/s, mean gradient ≥40 mm Hg, aortic valve area ≤1.0 cm2. Low-flow variants: D2 (AVA ≤1.0 cm2, Vmax <4 m/s or mean gradient <40 mm Hg, LVEF <50%) and D3 (paradoxical low flow; LVEF ≥50%, stroke volume index <35 ml/m2). Very severe AS means Vmax ≥5.0 m/s. ^[1][7]

Medical therapy. None slows stenosis progression; treat hypertension and comorbidities, and relieve symptoms. No routine vasodilator or statin therapy alters the valve. ^[5]

When to intervene. ^[1][5][7]

• Symptomatic severe AS (Stage D) or LV systolic dysfunction (Class I).
• Asymptomatic: at low surgical risk with decreasing exercise tolerance or an exercise fall in systolic blood pressure of ≥10 mm Hg; very severe AS (Vmax ≥5.0 m/s); BNP >3 times normal; or Vmax increasing by ≥0.3 m/s per year (Class IIa). Intervention can be considered if LVEF falls progressively to <60% on ≥3 serial studies (Class IIb).
• SAVR is preferred if age <65 years or life expectancy >20 years; TAVI if feasible in age >80 or life expectancy <10 years; shared decision making between ages 65 and 80; TAVI in high or prohibitive surgical risk with predicted survival >12 months. ^[1]
• Balloon aortic valvotomy is a bridge only, for haemodynamically unstable patients. ^[5]

Follow-up (echo). Mild AS every 3-5 years, moderate every 1-2 years, severe every 6-12 months. ^[5][7]

Cause. Leaflet disease (degenerative, rheumatic, endocarditis) or aortic root/ascending aorta disease (bicuspid valve, hypertension, Marfan, dissection).

Pathophysiology. Volume overload leads to eccentric hypertrophy and LV dilatation; LV function preserved for years then falls.

Symptoms/signs. Exertional dyspnoea, orthopnoea, a collapsing (water-hammer) pulse, a wide pulse pressure, and an early diastolic decrescendo murmur at the left sternal edge; an Austin Flint mid-diastolic rumble may be heard.

Investigations/grading. TTE quantifies regurgitant volume/volume fraction, chamber size and LV function. Severe AR justifies intervention triggers; the guides disagree on thresholds: AHA/ACC 2020 uses LVEF ≤55% (Stage C2), whereas ESC/EACTS uses LVEF <50%. ^[1][4][5]

Medical therapy. Control hypertension; losartan plus atenolol reduces aortic root dilatation in Marfan syndrome. ^[5] No medical therapy reverses AR.

When to intervene. Symptomatic patients regardless of LV function (Class I). Asymptomatic: LVEF ≤55% or LVESD >50 mm (or indexed LVESD >25 mm/m2) (AHA), or LVEF <50% / LVESD >50 mm / >25 mm/m2 (ESC 2021). Surgery is also indicated when CABG or ascending aorta/other valve surgery is performed. Valve-sparing aortic root replacement in young patients with root dilatation; ascending aortic surgery when aortic diameter ≥50 mm in Marfan. ^[1][4][5]

Follow-up (echo). Mild 3-5 years, moderate 1-2 years, severe 6-12 months. ^[5]

Cause. Usually rheumatic, especially in low- and middle-income countries; degenerative mitral annular calcification (MAC) is increasingly common in older adults in high-income countries. ^[2][5] Eighty percent of rheumatic MS occurs in women. ^[2]

Pathophysiology. Narrowing of the mitral valve obstructs flow from left atrium to left ventricle, raising left atrial pressure; this leads to pulmonary congestion and atrial fibrillation. The left ventricle is usually underfilled and normal.

Symptoms/signs. Exertional dyspnoea, orthopnoea, haemoptysis, palpitations; a low-pitched mid-diastolic rumble at the apex with an opening snap. In RHD, chordal fusion and commissural fusion rather than leaflet calcification dominate in the young.

Investigations/grading (echo). Severe rheumatic MS: mitral valve area ≤1.5 cm2 and/or pressure half-time ≥150 ms, typically mean gradient >5-10 mm Hg. ^[2] MAC-type MS is not benefited by balloon commissurotomy because stenosis results from annular calcification encroaching on the leaflet bases. ^[2]

Medical therapy. Diuretics for congestion, heart-rate slowing agents (beta-blockers, calcium-channel blockers, digoxin) to lengthen diastolic filling in atrial fibrillation, and anticoagulation when AF or thrombus is present. No drug reverses stenosis.

When to intervene. In symptomatic severe rheumatic MS with favourable morphology and less than moderate MR and no left atrial appendage thrombus, percutaneous mitral balloon commissurotomy (PMBC/PMC) is recommended at a Comprehensive Valve Centre (Class I/AHA); it is also recommended for suitable cases in the ESC guidance. ^[2][4] Surgery (repair or replacement) if balloon is not feasible, or in calcific MS where balloon is not beneficial, after careful risk-benefit consideration. ^[2][4]

Pregnancy. Valve intervention before conception is reasonable in severe asymptomatic rheumatic MS (valve area ≤1.5 cm2) with favourable morphology. ^[3]

Cause. Degenerative valve disease (MVP, flail leaflet), rheumatic disease, endocarditis.

Pathophysiology and presentation. Volume overload of the left atrium and ventricle; symptoms of heart failure; a holosystolic murmur at the apex radiating to the axilla.

Grading and management. Comprehensive TTE/TEE assessment of mechanism and MR severity. Surgery (repair preferred over replacement) for symptomatic severe primary MR; in asymptomatic patients surgery is recommended if LVEF ≤60% and/or LV end-systolic diameter ≥40 mm (Class I), or if MR is severe with a likely durable repair and low surgical risk. TEER is an option if surgical risk is high or prohibitive and anatomy is favourable. ^[2][5] Additional triggers mentioned in the literature include LA volume ≥60 ml/m2, systolic pulmonary pressure >50 mm Hg and atrial fibrillation. ^[5]

MVP occurs in 2 to 4% of people. ^[5] Most have a benign course, but advanced myxomatous degeneration (bi-leaflet prolapse, mitral annular disjunction) carries an excess of ventricular arrhythmias and sudden cardiac death, requiring careful monitoring even when repair is not indicated. See ventricular arrhythmias. ^[5]

Cause. Left ventricular or atrial disease (ischaemia, cardiomyopathy, atrial remodelling). Regurgitation is a consequence, not the primary disease.

Management. Guideline-directed medical therapy for heart failure is the mainstay and often reduces MR. ^[2][5] For severe secondary MR with LVEF 20-50%, LV end-systolic diameter ≤70 mm, and pulmonary artery systolic pressure <70 mm Hg persisting after optimisation, TEER is reasonable if anatomy is favourable (Class IIa, AHA). Surgery is reasonable concomitant with CABG. ^[2] For atrial (AF-related) annular dilatation with preserved LV function, mitral valve surgery may be considered if symptoms persist after HF optimisation and arrhythmia management. ^[2]

ESC/EACTS 2021 emphasises surgery or intervention in severe secondary MR only for patients who remain symptomatic despite GDMT. ^[4]

Cause. About 95% is secondary: annular dilatation from RV or right atrial dilatation, pulmonary hypertension, or atrial arrhythmia; primary causes include endocarditis, prolapse and congenital disease. ^[5]

Presentation. Fatigue, peripheral oedema, hepatomegaly, and a systolic murmur at the left lower sternal edge that increases on inspiration.

Management. Diuresis and treatment of the underlying cause (heart failure, pulmonary hypertension, arrhythmia). ^[2][5] For severe primary TR with right heart failure symptoms, isolated tricuspid surgery can improve symptoms and reduce hospitalisations (Class IIa). In asymptomatic severe primary TR, surgery may be considered if RV dilatation or dysfunction develops (Class IIb). For secondary TR, concomitant tricuspid surgery is indicated if TR is severe (Class I) during left-sided valve surgery, and considered if the annulus is dilated >4.0 cm or right heart failure has occurred (Class IIa). ^[2]

Usually rheumatic, often combined with MS and aortic disease. Surgical intervention is recommended for symptomatic severe tricuspid stenosis (and for any severe tricuspid stenosis undergoing left-sided valve surgery). ^[4]

Most isolated pulmonary valve disease is congenital stenosis; regurgitation occurs after correction of tetralogy of Fallot or from pulmonary hypertension. Presentation is of right ventricular pressure or volume overload. Balloon valvotomy is used for congenital stenosis in many centres. Surgical replacement is reserved for severe, symptomatic, or free regurgitation with right ventricular enlargement. There are limited source-supported specifics here; each case needs specialist assessment.

• Secondary MR: heart-failure guideline-directed medical therapy is first-line and can reduce regurgitation. ^[2][5]
• TR: diuretics and treatment of pulmonary hypertension/rhythm disturbance. ^[2][5]
• MS: diuretics and rate control; anticoagulation for AF; beta-blockers limit exercise pulmonary pressures in some patients.
• AS and AR: no medical therapy reverses the valve lesion. ^[5]
• Hypertension should be treated in patients with AS or regurgitant lesions, without excessive afterload reduction in severe AS. Validate with local practice.
• Anticoagulation: mechanical valves need a vitamin K antagonist (VKA); most native valve disease with AF does not require VKA unless there is rheumatic mitral stenosis or a mechanical valve. ^[1][9]

Prosthetic follow-up. After valve replacement, TTE is obtained post-procedure, then surveillance imaging at 5 and 10 years, then annually; with altered symptoms, additional imaging (TEE, fluoroscopy, CT) is used. For a bioprosthetic TAVI, annual TTE is reasonable. ^[3]

• Mechanical AVR with risk factors (hypercoagulable state, LV dysfunction, prior thromboembolism, older-generation prosthesis): INR 3.0.
• Mechanical bileaflet/current-generation single-tilting disk AVR with no risk factors: INR 2.5.
• Mechanical On-X AVR with no risk factors: INR 1.5-2.0, starting 3 months after surgery, plus aspirin 75-100 mg daily.
• Mechanical mitral valve replacement: INR 3.0.
• Bioprosthetic TAVI/SAVR or mitral valve replacement: aspirin 75-100 mg is reasonable; anticoagulation with VKA to INR 2.5 is reasonable for 3-6 months postoperatively.
• Direct thrombin inhibitors and anti-Xa DOACs are contraindicated with mechanical valves (Class III). ^[3][9]

Bridging: a mechanical bileaflet aortic valve without other risk factors does not require bridging; mechanical AVR with risk factors, older-generation mechanical AVRs and mechanical mitral valve replacements all need bridging anticoagulation. ^[3]

Women with severe valve disease considering pregnancy should receive pre-pregnancy counselling and be followed at a tertiary centre with a dedicated Heart Valve Team. ^[3]

• Valve intervention is reasonable before conception in asymptomatic severe rheumatic MS (valve area ≤1.5 cm2) or severe AS (aortic velocity ≥4.0 m/s or mean gradient ≥40 mm Hg); percutaneous mitral balloon commissurotomy with favourable morphology; mitral repair where suitable. ^[3]
• During pregnancy, intervention is reasonable for refractory NYHA class III-IV symptoms. ^[3]
• Mechanical valves cause high-risk pregnancies. Warfarin in the first trimester gives the lowest maternal complication rate but the highest fetal loss/malformation risk when the dose exceeds 5 mg/day. If warfarin <5 mg/day suffices, continuation for all three trimesters is reasonable; if >5 mg/day is needed, dose-adjusted low-molecular-weight heparin (LMWH) is used in the first trimester (anti-Xa target 0.8-1.2 U/ml 4-6 hours after dose) or IV unfractionated heparin (aPTT 2 times control), followed by warfarin later, or LMWH throughout. Direct oral anticoagulants are not recommended. ^[3]
• At least 1 week before delivery, switch to LMWH or IV heparin; switch to IV heparin ≥36 hours before delivery; stop heparin ≥6 hours before vaginal delivery. ^[3]

Current practice limits IE prophylaxis to the highest-risk cardiac conditions and to procedures that manipulate gingival tissue or break mucosa. ^[8] Conditions qualifying (per the AHA limited list):

• Prosthetic cardiac valve or prosthetic material used for valve repair
• Previous IE
• Congenital heart disease (unrepaired cyanotic lesions including shunts/conduits; completely repaired with prosthetic material in the first 6 months; residual defects at the site of repair)
• Cardiac transplant recipients who develop valvulopathy ^[8]

Regimen for dental procedures (single dose 30-60 minutes before): oral ampicillin 2 g in adults (50 mg/kg in children); for those unable to take oral medication ampicillin 2 g IM/IV or cefazolin/ceftriaxone 1 g IM/IV. Clindamycin, cefalexin, azithromycin or clarithromycin are alternatives in penicillin allergy. ^[8] Prophylaxis is not recommended for most other patients, including those with rheumatic heart disease without prosthetic material. ^[6]

Recurrent rheumatic fever worsens valve damage, so continuous antimicrobial prophylaxis is used after documented acute rheumatic fever or established RHD. ^[6]

• Penicillin G benzathine (intramuscular): 1,200,000 units every 4 weeks (every 3 weeks in high-risk situations) for patients weighing >27 kg; 600,000 units for those <27 kg. Oral alternatives: penicillin V 250 mg twice daily or sulfadiazine 1 g daily (>27 kg) / 0.5 g (<27 kg). ^[6]
• Duration (AHA): 10 years or until age 40 (whichever is longer) after carditis with residual valvular disease; 10 years or until age 21 without residual disease; 5 years or until age 21 without carditis. Lifelong prophylaxis may be needed. ^[2]

• Native valve disease (AHA style): mild 3-5 years, moderate 1-2 years, severe 6-12 months. ^[5][7]
• Prosthetic valves: TTE post-procedure, then follow-up at 5 and 10 years and annually thereafter; annual TTE is reasonable after TAVI. ^[3]
• RHD in endemic settings: regular clinical and echocardiographic review; secondary prophylaxis continued per above. ^[6]

• 2020 AHA/ACC (summarised in references 1-3) sets the Stages A-D system and the lesion-specific intervention triggers described above, including the valve-choice algorithm (SAVR versus TAVI) by age/life expectancy, INR targets and pregnancy rules.
• 2021 ESC/EACTS (reference 4) agrees on most points but differs in places that matter: it generally intervenes on severe secondary MR only when symptoms persist despite optimal therapy; it uses a lower LVEF threshold for surgery in AR (LVEF <50%, not ≤55%); it recommends PMC for suitable symptomatic MS; and it favours early surgery in symptomatic AS and in fit asymptomatic patients with systolic dysfunction. The choice between following lines should be documented and consistent; both are current and well respected. ^[4]

Cross-checks such as the ACC comparisons confirm that for severe AS both guidelines largely agree, differing in details such as the LVEF threshold for asymptomatic disease and exercise testing. ^[1]

RHD is the dominant cause of valve disease in Africa and other low-resource settings, presenting in teenagers and young adults. ^[5] Priorities there: ^[5][6]

• Treat streptococcal pharyngitis promptly (primary prevention) and maintain benzathine prophylaxis (secondary prevention).
• Echocardiography is the single most useful diagnostic tool; a focused echo can confirm rheumatic MS or MR even where specialist cardiology is unavailable.
• Balloon mitral commissurotomy is highly cost-effective for suitable rheumatic MS; surgery for calcific or combined disease.
• Mechanical valves carry a lifelong anticoagulation requirement that is hard to sustain where monitoring is limited; bioprostheses may be preferred in some women of childbearing age, with shared decisions.
• Infective endocarditis remains common; attention to dental hygiene is a major preventive effort. ^[8]

Complications include heart failure, atrial fibrillation and stroke (especially MS and prosthetic valves), endocarditis, arrhythmias, and sudden death in severe or untreated disease. Prognosis after timely valve intervention is generally good and approaches that of the general population for many lesions; delayed intervention reduces survival. ^[5]

• Older adults: degenerative calcific disease; assess frailty, comorbidity and life expectancy before intervention. ^[1]
• Children and young adults: rheumatic and congenital disease; think of rheumatic fever in any child with a murmur in an endemic area.
• Pregnancy: see the dedicated section above; multidisciplinary care at a tertiary centre. ^[3]

• Valve disease is a mechanical problem: stenosis is a pressure load, regurgitation a volume load.
• No drug cures a narrowed or leaking valve; interventions are definitive for severe disease.
• TTE grades severity; staging A-D guides timing.
• Severe symptomatic AS (D1/D2/D3) needs intervention; SAVR under 65, TAVI over 80, shared decisions between.
• AR intervention triggers are symptom onset, LVEF fall (≤55% AHA / <50% ESC) or significant LV dilation; guidelines differ.
• Rheumatic MS: PMC/PMBC for suitable valves; calcific MS needs careful surgical judgement.
• Primary MR: repair preferred; surgery at LVEF ≤60% or LVESD ≥40 mm; TEER an option at high risk.
• Secondary MR: heart-failure therapy first; TEER in selected patients.
• TR: usually secondary; treat the cause; consider surgery with left-sided surgery or severe symptomatic disease.
• Mechanical valves: VKA only, with INR targets by valve; pregnancy requires a structured anticoagulation plan.
• IE prophylaxis is now limited to the highest-risk conditions; ampicillin 2 g before dental procedures in adults.
• Rheumatic fever secondary prevention with benzathine penicillin, with duration by carditis/residual disease.
• Follow-up intervals: mild 3-5 years, moderate 1-2 years, severe 6-12 months; prosthetics 5 and 10 years then annually.

• Heart failure
• ECG interpretation
• Cardiac physical examination
• Atrial fibrillation and flutter
• Ventricular arrhythmias
• Bradyarrhythmias and AV block
• Supraventricular tachycardias
• Approach to tachyarrhythmias
• Heart failure with preserved and reduced ejection fraction; acute pericarditis; STEMI; NSTEMI; aortic dissection; hypertensive crises.