What is the treatment of rheumatic heart disease?

Treatment of Rheumatic Heart Disease

All patients with rheumatic heart disease should receive benzathine penicillin G 1.2 million units intramuscularly every 4 weeks as lifelong secondary prophylaxis, combined with guideline-directed medical therapy for heart failure when left ventricular dysfunction develops, anticoagulation for atrial fibrillation, and timely surgical intervention for severe symptomatic valve disease. 1

Secondary Antibiotic Prophylaxis (Cornerstone of Treatment)

First-Line Regimen

• Benzathine penicillin G 1.2 million units intramuscularly every 4 weeks is the gold standard with Class I, Level A evidence and is approximately 10-fold more effective than oral regimens 2, 3, 4
• For high-risk patients (prior recurrence despite adherence, ongoing high streptococcal exposure, teachers, daycare workers, healthcare workers in endemic areas), shorten the injection interval to every 3 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 preferred second-line option for penicillin-allergic patients 1, 3
• Sulfadiazine 1 g orally once daily (0.5 g for patients ≤27 kg) is an alternative for penicillin allergy 1, 3
• Macrolide or azalide antibiotics may be used only when allergic to both penicillin and sulfadiazine, but must be avoided in patients taking CYP3A inhibitors (azole antifungals, HIV protease inhibitors, certain SSRIs) 1, 2

Duration of Secondary Prophylaxis

Clinical Scenario	Duration (whichever is longer)
Rheumatic fever with carditis AND residual valvular disease	10 years after last attack OR until age 40 [1,2]
Rheumatic fever with carditis WITHOUT residual valvular disease	10 years after last attack OR until age 21 [1,2]
Rheumatic fever WITHOUT carditis	5 years after last attack OR until age 21 [1,2]
High-risk occupational/community exposure	Lifelong prophylaxis [1,2]

Critical Prophylaxis Principles

• Before initiating long-term prophylaxis, administer a full therapeutic course of penicillin to eradicate residual group A Streptococcus, even if throat culture is negative 1, 2, 5
• Secondary prophylaxis must continue after valve replacement or repair because surgery does not eliminate the risk of recurrent acute rheumatic fever 1, 2, 5
• Never discontinue prophylaxis prematurely, even if the patient feels well, has normal echocardiogram, or has undergone valve surgery 2, 3

Medical Management of Valvular Heart Disease

Heart Failure Therapy

• When left ventricular systolic dysfunction develops, institute standard guideline-directed medical therapy: diuretics, ACE inhibitors or ARBs, beta-blockers, aldosterone antagonists, and sacubitril/valsartan 1, 2, 3
• Avoid abrupt blood pressure reduction in stenotic valve lesions (mitral or aortic stenosis) as this can precipitate hemodynamic collapse 1, 2
• Beta-blockers are essential for heart rate control, especially in mitral stenosis where diastolic filling time is critical 2
• Digoxin may be added for additional rate control in atrial fibrillation when beta-blockers alone are insufficient 2

Anticoagulation Strategy

• Anticoagulation should be considered for all women with rheumatic heart disease who have atrial fibrillation 2
• Anticoagulation is also reasonable in patients in sinus rhythm who have very severe left atrial dilatation, spontaneous echo contrast, or concurrent heart failure 2

Surgical and Percutaneous Intervention

Timing of Intervention

• All patients with symptomatic severe rheumatic mitral stenosis should be evaluated for percutaneous mitral balloon commissurotomy (PMBC) or mitral valve surgery within 3 months of diagnosis 2
• In asymptomatic patients with severe rheumatic mitral stenosis (mitral valve area ≤1.5 cm²), PMBC at a comprehensive valve center is reasonable 2
• Women with moderate-to-severe mitral stenosis planning pregnancy should undergo PMBC before conception if symptomatic or if stenosis is clinically significant 2
• If percutaneous intervention is required during pregnancy, PMBC should be performed only after the 20th gestational week and exclusively in experienced centers with multidisciplinary teams 2

Surgical Considerations

• Surgery with mitral valve replacement or repair is indicated in cases with intractable hemodynamics due to mitral regurgitation 6
• Valvular regurgitation, not myocarditis, is the cause of congestive heart failure in active rheumatic carditis 6

Infective Endocarditis Prophylaxis

When IE Prophylaxis IS Indicated

Antibiotic prophylaxis before dental procedures that manipulate gingival tissue, periapical region, or perforate oral mucosa is reasonable (Class IIa) ONLY for patients with: 1, 2

• Prosthetic cardiac valves (including transcatheter-implanted prostheses and homografts)
• Prosthetic material used for valve repair (annuloplasty rings, chords, clips)
• Previous infective endocarditis
• Unrepaired cyanotic congenital heart disease or repaired disease with residual shunts

When IE Prophylaxis IS NOT Indicated

• Routine IE prophylaxis is NOT recommended for rheumatic heart disease alone 2, 3, 5
• Antibiotic prophylaxis is NOT recommended for non-dental procedures (transesophageal echocardiography, esophagogastroduodenoscopy, colonoscopy, cystoscopy) in the absence of active infection 1, 2
• Maintaining optimal oral hygiene remains the most important preventive measure against infective endocarditis 1, 2, 3

Special Consideration for Patients on Penicillin Prophylaxis

• For patients receiving benzathine penicillin G prophylaxis who require IE prophylaxis for dental procedures, use an alternative antibiotic (e.g., macrolide) rather than penicillin, as oral α-hemolytic streptococci likely have developed penicillin resistance 5

Additional Preventive Measures

Vaccinations and Lifestyle

• Influenza and pneumococcal vaccinations should be administered to all patients with rheumatic heart disease according to standard adult vaccination schedules 1, 2, 3
• Regular aerobic exercise is encouraged to improve cardiovascular fitness in patients with asymptomatic valvular heart disease 1, 2, 3
• Resistive training with small free weights or repetitive isolated muscle training may be used to strengthen individual muscle groups, but avoid heavy isometric repetitive training 1

Cardiovascular Risk Factor Management

• Standard guideline-directed medical therapy for cardiac risk factors (hypertension, diabetes mellitus, hyperlipidemia) should not be neglected 1
• Heart-healthy lifestyle factors (exercising, consuming a healthy diet, not smoking, maintaining normal body weight) apply equally to patients with valvular heart disease 1

Common Pitfalls to Avoid

Prophylaxis Errors

• Do not stop prophylaxis at arbitrary age cutoffs without evaluating individual risk factors such as ongoing streptococcal exposure, severity of valvular disease, and time since last rheumatic attack 2, 3
• Do not assume valve replacement eliminates rheumatic fever risk, as patients remain susceptible to group A streptococcus infection and recurrent acute rheumatic fever 5
• Do not use oral antibiotics as first-line prophylaxis when intramuscular benzathine penicillin G is available, as oral regimens are significantly less effective 4

Safety Considerations for High-Risk Patients

• Recent evidence suggests that patients with severe mitral stenosis, aortic stenosis, aortic insufficiency, or decreased left ventricular systolic dysfunction may be at elevated risk of cardiovascular compromise following benzathine penicillin G injections 7
• For these elevated-risk patients, oral prophylaxis should be strongly considered as the risk of adverse reaction to intramuscular benzathine penicillin G may outweigh its theoretical benefit 7
• Implement a multifaceted strategy for vasovagal risk reduction in all patients with rheumatic heart disease receiving benzathine penicillin G 7

Evidence Supporting Long-Term Prophylaxis

Effectiveness Data

• Secondary prophylaxis is efficient in reducing disease recurrence, heart damage, and cardiac impairment 8
• In a Brazilian cohort followed from 1986-2018, lesions on mitral and aortic valves showed regression in 69.9% and 48.7% of patients, respectively, with regular prophylaxis 8
• No patients undergoing regular prophylaxis presented progression of rheumatic cardiac disease 8
• Patients with disease progression were associated with noncompliance to secondary prophylaxis 8, 6
• Intramuscular benzathine benzylpenicillin likely reduces recurrence of rheumatic fever substantially (0.1% versus 1% with oral antibiotics) 4