What is the treatment for a patient with rheumatic heart disease?

Treatment of Rheumatic Heart Disease

All patients with rheumatic heart disease require long-term antibiotic prophylaxis with intramuscular benzathine penicillin G 1.2 million units every 4 weeks (or every 3 weeks in high-risk situations) to prevent recurrent acute rheumatic fever, combined with guideline-directed medical therapy for cardiac complications and timely valve intervention when indicated. 1, 2, 3

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 1, 3, 4
• For high-risk patients (those with severe valvular disease, high streptococcal exposure risk, or history of recurrent rheumatic fever despite prophylaxis), administer every 3 weeks instead of every 4 weeks to maintain protective penicillin levels 1, 3

Alternative Regimens for Penicillin Allergy

• Oral penicillin V 250 mg twice daily 1, 4
• Sulfadiazine 1 gram orally once daily (0.5 gram for patients ≤27 kg) 1, 3
• Macrolide antibiotics (dose varies), but avoid in patients taking cytochrome P450 3A inhibitors (azole antifungals, HIV protease inhibitors, some SSRIs) due to drug interactions 1, 3

Duration of Prophylaxis (Critical Decision Point)

The duration depends on disease severity and must be individualized based on these specific criteria 1:

• With persistent valvular disease: 10 years after last attack OR until age 40 years (whichever is longer), with consideration for lifelong prophylaxis in high-risk patients 1, 3
• With carditis but no residual valvular disease: 10 years after last attack OR until age 21 years (whichever is longer) 1
• Without carditis: 5 years after last attack OR until age 21 years (whichever is longer) 1

Critical caveat: Prophylaxis must continue even after valve replacement surgery, as surgery does not eliminate the risk of recurrent acute rheumatic fever 1, 3

Medical Management of Cardiac Complications

Heart Failure and LV Systolic Dysfunction

When valve intervention is not immediately feasible or declined, apply standard guideline-directed medical therapy 1, 2:

• Diuretics for volume management 1, 2
• ACE inhibitors or ARBs for afterload reduction 1, 2
• Beta-blockers for rate control and cardiac protection 1, 2
• Aldosterone antagonists when indicated 1, 2
• Sacubitril/valsartan in appropriate candidates 1, 2
• Biventricular pacing if meeting criteria for cardiac resynchronization therapy 1

Important warning: In patients with stenotic valve lesions (especially mitral or aortic stenosis), avoid abrupt lowering of blood pressure as this can precipitate hemodynamic collapse 1

Atrial Fibrillation Management

• Anticoagulation for stroke prevention according to standard guidelines 1, 2
• Rate control with beta-blockers or calcium channel blockers 2

Valve Intervention (Definitive Treatment for Severe Disease)

Indications for Intervention

Evaluate all patients with symptomatic severe rheumatic mitral stenosis (mitral valve area ≤1.5 cm²) for intervention within 3 months of diagnosis 2

Percutaneous Mitral Balloon Commissurotomy (PMBC)

• Preferred first-line 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 2
• Long-term outcomes: 70-80% of patients with good initial PMBC results remain free of recurrent symptoms at 10 years 2

Surgical Valve Intervention

Indicated when 2:

• Valve anatomy is unfavorable for PMBC
• PMBC has failed
• Moderate-to-severe tricuspid regurgitation requiring repair
• Significant mitral regurgitation (≥2+)

Critical principle: Medical therapy alone is not a substitute for definitive valve intervention in symptomatic patients with severe disease—delaying intervention increases morbidity and mortality 2

Additional Preventive Measures

Infective Endocarditis Prevention

• Maintain optimal oral health—this is the single most important preventive measure 1, 3
• Antibiotic prophylaxis before dental procedures involving manipulation of gingival tissue, periapical region of teeth, or perforation of oral mucosa is reasonable for patients with rheumatic heart disease at highest risk 1

Vaccinations

• Influenza vaccination annually 1, 3
• Pneumococcal vaccination per standard recommendations 1, 3

Lifestyle Modifications

• Regular aerobic exercise to improve cardiovascular fitness in asymptomatic patients 1, 3
• Avoid heavy isometric repetitive training, but resistive training with small free weights is acceptable 1

Echocardiographic Monitoring Schedule

Surveillance frequency based on disease severity 1, 2, 3:

• Mild disease: Every 3-5 years 1, 2
• Moderate disease: Every 1-2 years 1, 2
• Severe disease or dilating LV: Every 6-12 months 1, 2

Special Considerations in Pregnancy

• Pre-pregnancy evaluation mandatory for women with moderate-severe rheumatic heart disease 2
• Consider interventional therapy before pregnancy in asymptomatic patients with severe rheumatic mitral stenosis 2
• During pregnancy: beta-blockers, diuretics, and anticoagulation as needed 2

Critical Pitfalls to Avoid

• Never discontinue secondary prophylaxis prematurely, even if the patient feels well or has undergone valve surgery 1, 3
• Never delay valve intervention in symptomatic patients with severe disease—medical therapy alone is inadequate 2
• Never abruptly lower blood pressure in patients with stenotic lesions 1
• Never overlook pregnancy as a high-risk period requiring specialized management 2
• Never neglect regular echocardiographic follow-up, as disease progression can be insidious 2, 3
• 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 1, 3