Management and Treatment of Rheumatic Heart Disease
Secondary Antibiotic Prophylaxis: The Cornerstone of RHD Management
All patients with rheumatic heart disease require long-term antibiotic prophylaxis to prevent recurrent rheumatic fever and progression of valvular damage, regardless of whether they have undergone valve replacement surgery. 1, 2
First-Line Prophylactic Regimen
Benzathine penicillin G 1.2 million units intramuscularly every 4 weeks is the gold standard prophylaxis regimen with Class I, Level A evidence from the American Heart Association. 1, 2, 3
Intramuscular benzathine penicillin G is approximately 10 times more effective than oral antibiotics in preventing rheumatic fever recurrence (0.1% recurrence rate versus 1% with oral antibiotics). 4, 5
For high-risk patients (those with recurrence despite adherence, severe valvular disease, 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, 5
The injection must be given by deep intramuscular injection in the upper outer quadrant of the buttock (dorsogluteal) or ventrogluteal site, administered slowly and steadily to prevent needle blockage. 3
Alternative Regimens for Penicillin Allergy
Penicillin V 250 mg orally twice daily is the second-line option for patients unable to receive intramuscular injections or with penicillin allergy concerns. 1, 2
Sulfadiazine 1 gram orally once daily (or 0.5 gram once daily for patients weighing ≤27 kg) is recommended for penicillin-allergic patients. 1, 2, 5
Macrolide or azalide antibiotics (such as erythromycin) may be used for patients allergic to both penicillin and sulfadiazine, but avoid macrolides in patients taking cytochrome P450 3A inhibitors (azole antifungals, HIV protease inhibitors, some SSRIs) due to dangerous drug interactions. 1, 2, 6
Duration of Prophylaxis Based on Disease Severity
The duration of secondary prophylaxis depends on the presence and severity of cardiac involvement:
For patients with rheumatic fever and persistent valvular disease (residual RHD): Continue prophylaxis for 10 years after the last attack OR until age 40 years, whichever is longer. 1, 2
Consider lifelong prophylaxis for patients at high risk of group A streptococcus exposure (teachers, daycare workers, healthcare workers, those living in crowded conditions). 1, 2
For patients with rheumatic fever and carditis but no residual heart disease: Continue prophylaxis for 10 years after the last attack OR until age 21 years, whichever is longer. 1, 5
For patients with rheumatic fever without carditis: Continue prophylaxis for 5 years after the last attack OR until age 21 years, whichever is longer. 1, 5
Critical Management Point: Prophylaxis After Valve Surgery
Secondary antibiotic prophylaxis must continue even after valve replacement surgery, as valve replacement does not eliminate the risk of recurrent acute rheumatic fever from group A streptococcus infection. 2, 1 This is a common pitfall—never discontinue prophylaxis prematurely based on surgical intervention alone. 2
Medical Management of Cardiac Complications
Guideline-Directed Medical Therapy for Heart Failure
When left ventricular systolic dysfunction develops in RHD patients, apply standard guideline-directed medical therapy including:
- Diuretics for volume management 1, 5
- ACE inhibitors or ARBs for afterload reduction 1, 5
- Beta-blockers for rate control and mortality benefit 1, 5
- Aldosterone antagonists for advanced heart failure 1, 5
- Sacubitril/valsartan when indicated per heart failure guidelines 1, 5
Avoid abrupt lowering of blood pressure in patients with stenotic valve lesions (particularly mitral stenosis or aortic stenosis), as this can precipitate hemodynamic collapse. 1, 5
Anticoagulation for Atrial Fibrillation
- Patients with RHD who develop atrial fibrillation require anticoagulation for stroke prevention, following standard anticoagulation guidelines for valvular heart disease. 1
Interventional Management: Timing of Valve Procedures
Indications for Valve Intervention
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. 7
PMBC is the preferred intervention for patients with favorable valve morphology (mobile, relatively thin leaflets free of calcium, without significant subvalvular fusion) and less than 2+ mitral regurgitation in the absence of left atrial thrombus. 7
Long-term outcomes demonstrate that 70-80% of patients with good initial PMBC results remain free of recurrent symptoms at 10 years. 7
Surgical valve replacement is indicated when:
Never delay valve intervention in symptomatic patients with severe disease, as medical therapy alone is not a substitute for definitive treatment and delays increase morbidity and mortality. 7
Infective Endocarditis Prophylaxis Considerations
Current guidelines do NOT recommend routine endocarditis prophylaxis for rheumatic heart disease alone, unless the patient has prosthetic valves, prosthetic material used in valve repair, or previous infective endocarditis. 1, 2, 5
However, patients with RHD (including those receiving benzathine penicillin G prophylaxis) should receive amoxicillin prophylaxis before undergoing high-risk dental or surgical procedures involving manipulation of gingival tissue or perforation of oral mucosa. 6, 5
Use an agent other than penicillin (such as clindamycin) for endocarditis prophylaxis in patients already receiving penicillin for rheumatic fever prophylaxis, or in those with immediate penicillin hypersensitivity. 5, 6
Maintaining optimal oral health remains the most important preventive measure against infective endocarditis in all patients with rheumatic heart disease. 1, 2
Additional Preventive Measures and Lifestyle Modifications
Vaccinations
- Administer influenza and pneumococcal vaccinations according to standard recommendations for all patients with RHD. 1, 5
Exercise and Cardiovascular Fitness
Encourage regular aerobic exercise to improve cardiovascular fitness in patients with asymptomatic valvular heart disease. 1, 5
Heavy isometric repetitive training should be avoided as it increases LV afterload, but resistive training with small free weights or repetitive isolated muscle training may be used to strengthen individual muscle groups. 1
Cardiovascular Risk Factor Management
Standard guideline-directed medical therapy for cardiac risk factors (hypertension, diabetes mellitus, hyperlipidemia) should not be neglected in patients being evaluated for valvular heart disease. 1
Heart-healthy lifestyle factors (exercising, consuming a healthy diet, not smoking, maintaining a normal body size) apply equally to patients with RHD as to the general population. 1
Common Pitfalls to Avoid
Never discontinue secondary prophylaxis prematurely, even if the patient feels well, has undergone valve surgery, or reaches an arbitrary age cutoff without considering individual risk factors. 2, 7
Never assume that valve replacement eliminates the need for antibiotic prophylaxis—this is perhaps the most dangerous misconception in RHD management. 2, 1
Avoid inadequate anticoagulation monitoring in patients with atrial fibrillation or mechanical valves. 7
Recognize pregnancy as a high-risk period requiring specialized evaluation and management. 7
Do not neglect regular follow-up echocardiography: every 3-5 years for mild disease, every 1-2 years for moderate disease, and every 6-12 months for severe disease or when the left ventricle is dilating. 7
Adherence Strategies
Compliance with prophylactic antibiotics remains poor (approximately 30% in some studies), leaving patients at risk of avoidable and progressive heart damage. 8, 9
Strategies to improve adherence include: