Management of Rheumatic Heart Disease
Secondary Antibiotic Prophylaxis: The Cornerstone of Management
Benzathine penicillin G 1.2 million units intramuscularly every 4 weeks is the gold standard first-line prophylaxis for all patients with rheumatic heart disease, with Class I, Level A evidence from the American Heart Association and American College of Cardiology. 1, 2
Primary Prophylaxis Regimen
- Intramuscular benzathine penicillin G is approximately 10 times more effective than oral antibiotics in preventing recurrent acute rheumatic fever and progression of valve damage. 3
- For high-risk patients (those with severe valvular disease, previous recurrences despite adherence, or high streptococcal exposure risk), administer benzathine penicillin G every 3 weeks instead of every 4 weeks to maintain more consistent protective penicillin levels. 1, 2, 3
Alternative Regimens for Penicillin Allergy
- Oral penicillin V 250 mg twice daily is the second-line option for patients who cannot tolerate intramuscular injections or have non-severe penicillin hypersensitivity. 1, 2, 4
- Sulfadiazine 1 gram orally once daily (or 0.5 gram for patients weighing ≤27 kg) for patients allergic to penicillin. 1, 2
- Macrolide or azalide antibiotics (such as erythromycin or azithromycin) only for patients allergic to both penicillin and sulfadiazine, but never use macrolides in patients taking cytochrome P450 3A inhibitors (azole antifungals, HIV protease inhibitors, some SSRIs) due to dangerous drug interactions. 1, 2
Duration of Prophylaxis: Risk-Stratified Approach
The duration depends on disease severity and must be individualized based on cardiac involvement:
- Rheumatic fever WITH carditis AND persistent valvular disease: Continue prophylaxis for 10 years after last attack OR until age 40 years, whichever is LONGER. 1, 2, 3
- Rheumatic fever WITH carditis but NO residual heart disease: Continue for 10 years after last attack OR until age 21 years, whichever is longer. 1, 3
- Rheumatic fever WITHOUT carditis: Continue for 5 years after last attack OR until age 21 years, whichever is longer. 1, 3
- Consider lifelong prophylaxis for patients at persistently high risk of group A streptococcus exposure (healthcare workers, teachers, military personnel, those living in endemic areas). 1, 2, 3
Critical Management Point: Never Stop Prophylaxis After Valve Surgery
Secondary prophylaxis must continue even after valve replacement surgery, as valve replacement does not eliminate the risk of recurrent acute rheumatic fever from new group A streptococcus infections. 2, 3 This is a common and dangerous pitfall.
Medical Management of Cardiac Complications
Heart Failure and Left Ventricular Dysfunction
When left ventricular systolic dysfunction develops, apply guideline-directed medical therapy including:
- Diuretics for volume management 1, 3, 5
- ACE inhibitors or ARBs for afterload reduction 1, 3, 5
- Beta-blockers for rate control and mortality benefit 1, 3, 5
- Aldosterone antagonists when indicated 1, 3
- Sacubitril/valsartan for advanced heart failure 1, 3
Critical caveat: In patients with stenotic valve lesions (especially mitral or aortic stenosis), avoid abrupt lowering of blood pressure as this can precipitate hemodynamic collapse. 1, 3, 5
Atrial Fibrillation Management
- Anticoagulation is required for stroke prevention in patients with rheumatic heart disease and atrial fibrillation. 5
- Beta-blockers or other rate-control agents should be used to manage ventricular rate. 5
Valve Intervention: Timing and Modality
All patients with symptomatic severe rheumatic mitral stenosis (mitral valve area ≤1.5 cm²) should be evaluated for percutaneous mitral balloon commissurotomy (PMBC) or mitral valve surgery within 3 months of diagnosis. 5
Percutaneous Mitral Balloon Commissurotomy (PMBC)
- PMBC is the preferred intervention for patients with favorable valve morphology: mobile leaflets, relatively thin leaflets free of calcium, without significant subvalvular fusion, and less than 2+ mitral regurgitation in the absence of left atrial thrombus. 5
- Long-term outcomes are excellent: 70-80% of patients with good initial PMBC results remain free of recurrent symptoms at 10 years. 5
Surgical Valve Intervention
Surgical intervention is indicated when:
- Valve anatomy is unfavorable for PMBC 5
- PMBC has failed 5
- Patients have moderate-to-severe tricuspid regurgitation requiring repair 5
Never delay valve intervention in symptomatic patients with severe disease, as medical therapy alone is not a substitute for definitive treatment and delays increase mortality risk. 5
Infective Endocarditis Prophylaxis: A Nuanced Approach
Current guidelines do NOT recommend routine endocarditis prophylaxis for rheumatic heart disease alone. 2, 3 However, there are important exceptions:
When Endocarditis Prophylaxis IS Indicated
Antibiotic prophylaxis before dental procedures (involving manipulation of gingival tissue, periapical region of teeth, or perforation of oral mucosa) is reasonable for patients with: 1
- Prosthetic cardiac valves 1, 3
- Prosthetic material used for valve repair 1, 3
- Previous infective endocarditis 1, 3
Critical Point for Patients Already on Rheumatic Fever Prophylaxis
For patients receiving penicillin prophylaxis for rheumatic fever who require endocarditis prophylaxis for dental procedures, use an agent OTHER than penicillin (such as clindamycin or amoxicillin if recently treated with penicillin). 3, 6
The Most Important Preventive Measure
Maintaining optimal oral health remains the most important preventive measure against infective endocarditis in all patients with rheumatic heart disease, far more effective than antibiotic prophylaxis. 1, 2, 3
Additional Preventive Measures and Monitoring
Vaccinations
- Influenza and pneumococcal vaccinations should follow standard recommendations for all patients with rheumatic heart disease. 1, 3, 5
Exercise and Lifestyle
- Encourage regular aerobic exercise to improve cardiovascular fitness in patients with asymptomatic valvular heart disease. 1, 3
- Heart-healthy lifestyle factors (healthy diet, not smoking, maintaining normal body weight) apply equally to patients with rheumatic heart disease. 1
Echocardiographic Surveillance
Regular echocardiographic monitoring is essential: 5
- Every 3-5 years for mild disease
- Every 1-2 years for moderate disease
- Every 6-12 months for severe disease or when left ventricle is dilating
Common Pitfalls to Avoid
Never discontinue secondary prophylaxis prematurely, even if the patient feels well, has normal echocardiograms, or has undergone valve surgery. 2, 3, 5
Never stop prophylaxis at arbitrary age cutoffs without considering individual risk factors such as ongoing streptococcal exposure, severity of valvular disease, and time since last attack. 2
Before initiating long-term prophylaxis, always administer a full therapeutic course of penicillin to eradicate any residual group A streptococcus, even if throat culture is negative at diagnosis. 2, 3, 7
Never overlook pregnancy as a high-risk period for women with moderate-severe rheumatic heart disease; these patients should be evaluated before pregnancy and interventional therapy considered. 5
Avoid inadequate anticoagulation monitoring in patients with atrial fibrillation or mechanical valves. 5