What is the management and treatment for rheumatic heart disease affecting the valves?

Management of Rheumatic Heart Disease Valves

For patients with rheumatic heart disease affecting the valves, initiate lifelong intramuscular benzathine penicillin G (1.2 million units every 4 weeks) as the cornerstone of management, combined with guideline-directed medical therapy for heart failure when present, and timely surgical or percutaneous intervention for severe symptomatic valve disease. 1, 2

Secondary Antibiotic Prophylaxis: The Foundation of Management

Intramuscular benzathine penicillin G is superior to oral alternatives and must be continued indefinitely in patients with persistent valvular disease. 1, 2

• Administer benzathine penicillin G 1.2 million units intramuscularly every 4 weeks as first-line prophylaxis to prevent recurrent rheumatic fever, which would worsen existing valve damage 3, 1, 2

• Continue prophylaxis for at least 10 years after the last acute rheumatic fever episode OR until age 40 years, whichever is longer, in patients with persistent valvular disease 3, 2

• Consider lifelong prophylaxis for high-risk patients with severe valvular disease or those with ongoing exposure to group A streptococcus (teachers, healthcare workers, daycare workers) 3, 2

• For penicillin-allergic patients, use oral penicillin V 250 mg twice daily, sulfadiazine 1 g daily (0.5 g if weight ≤27 kg), or erythromycin as alternatives, though these have lower efficacy 1, 2, 4

Critical caveat: Recent evidence from the American Heart Association identifies that patients with severe mitral stenosis, aortic stenosis, aortic insufficiency, or reduced left ventricular function may experience cardiovascular compromise from intramuscular benzathine penicillin G injections—in these elevated-risk patients, strongly consider switching to oral prophylaxis despite its lower efficacy 5

Medical Management of Heart Failure and Valve Dysfunction

Implement standard heart failure therapy immediately when left ventricular dysfunction or symptoms develop. 1, 6

• Start loop diuretics (furosemide) for pulmonary congestion or peripheral edema 6

• Initiate ACE inhibitors or ARBs for afterload reduction, particularly beneficial in mitral and aortic regurgitation 1, 6

• Add beta-blockers for heart failure with reduced ejection fraction and for rate control in atrial fibrillation 6

• Consider aldosterone antagonists (spironolactone, eplerenone) for persistent symptoms despite ACE inhibitor and beta-blocker therapy 6

• Use sacubitril/valsartan in place of ACE inhibitors for patients with heart failure with reduced ejection fraction who remain symptomatic 6

• Add digoxin for symptom control, especially when atrial fibrillation is present 6

• Avoid abrupt blood pressure lowering in patients with severe aortic or mitral stenosis, as they are preload-dependent 3

Anticoagulation for Atrial Fibrillation

All patients with rheumatic heart disease and atrial fibrillation require anticoagulation with warfarin, not direct oral anticoagulants. 3, 6, 7

• Target INR of 2.5 (range 2.0-3.0) for patients with rheumatic mitral stenosis and atrial fibrillation 7

• Warfarin is specifically recommended over DOACs because the landmark trials establishing DOAC efficacy excluded patients with valvular atrial fibrillation (particularly rheumatic mitral stenosis) 7

• Add rate control with beta-blockers or digoxin to manage ventricular response 6

Surgical and Percutaneous Intervention Timing

Intervene surgically or percutaneously when patients develop symptoms with moderate-to-severe valve disease, or in asymptomatic patients with severe disease before pregnancy. 3, 1

• Percutaneous mitral balloon commissurotomy is the preferred intervention for rheumatic mitral stenosis when valve morphology is favorable (pliable, non-calcified leaflets without significant subvalvular fusion) 3, 1

• Mitral valve repair is superior to replacement when technically feasible, preserving native valve tissue and avoiding prosthetic valve complications 3

• Mitral valve replacement is indicated when commissurotomy fails, valve morphology is unfavorable, or significant mitral regurgitation coexists 3, 1

• Aortic valve replacement is required for severe symptomatic aortic stenosis or regurgitation; rheumatic aortic disease typically requires replacement rather than repair 3

• Perform coronary angiography before valve surgery in men over 40 years, postmenopausal women, or anyone with cardiovascular risk factors, left ventricular dysfunction, or suspected ischemia 3

• Operative mortality ranges from 2-8% depending on valve involved, whether isolated or combined with coronary bypass, and whether repair versus replacement 3

Endocarditis Prophylaxis

Patients with rheumatic heart disease require antibiotic prophylaxis only for high-risk dental procedures, unless already receiving benzathine penicillin G for rheumatic fever prophylaxis. 3, 1

• Use amoxicillin 2 g orally 30-60 minutes before dental procedures involving gingival manipulation or oral mucosa perforation 1, 4

• If the patient recently received penicillin or has penicillin allergy, use clindamycin 600 mg instead to avoid resistance 4

• Do not use additional endocarditis prophylaxis if the patient is already receiving regular benzathine penicillin G injections for rheumatic fever prophylaxis 2

• After prosthetic valve placement, endocarditis prophylaxis becomes mandatory for all high-risk procedures 3, 2

Pregnancy Considerations

Women with moderate-to-severe rheumatic valve disease must be evaluated and treated before conception. 1

• Perform percutaneous mitral balloon commissurotomy before pregnancy in asymptomatic women with severe rheumatic mitral stenosis to prevent maternal and fetal complications 1

• During pregnancy, use beta-blockers for rate control and symptom management, diuretics cautiously for volume overload, and anticoagulation (warfarin in first and third trimesters, heparin in second trimester) as needed 1

• Continue benzathine penicillin G prophylaxis throughout pregnancy 1, 2

Monitoring and Follow-Up

Echocardiographic surveillance frequency depends on disease severity and ventricular function. 1, 6

• Every 6-12 months for severe valve disease or when left ventricle is dilating 1, 6

• Every 1-2 years for moderate valve disease 6

• Every 3-5 years for mild valve disease 6

• Monitor for development of atrial fibrillation, heart failure symptoms, and progression of valve stenosis or regurgitation 1, 6

Additional Preventive Measures

• Maintain optimal oral hygiene with regular dental care to reduce endocarditis risk 3, 1

• Administer influenza and pneumococcal vaccinations to prevent respiratory infections that could precipitate heart failure 1, 6

• Ensure aseptic technique during all invasive procedures and catheter manipulations 3

Critical Pitfalls to Avoid

• Never discontinue secondary prophylaxis prematurely—even after valve surgery, patients remain susceptible to recurrent rheumatic fever 2

• Never use DOACs instead of warfarin for atrial fibrillation in rheumatic valve disease, as efficacy is unproven in this population 7

• Never delay intervention in symptomatic patients with severe valve disease, as mortality increases significantly with prolonged heart failure 3, 1

• Never overlook pregnancy as a high-risk period—hemodynamic changes can precipitate acute decompensation in women with moderate-to-severe stenotic lesions 1

• Never assume valve replacement eliminates the need for rheumatic fever prophylaxis—the underlying susceptibility to group A streptococcus persists 2