What are the management guidelines for rheumatic heart disease?

Management of Rheumatic Heart Disease

All patients with rheumatic heart disease require long-term secondary antibiotic prophylaxis with intramuscular benzathine penicillin G (1.2 million units every 4 weeks) as the cornerstone of management, combined with guideline-directed medical therapy for cardiac complications and timely valve intervention when indicated. 1

Secondary Antibiotic Prophylaxis

First-Line Regimen

• Intramuscular benzathine penicillin G (BPG) 1.2 million units every 4 weeks is the gold standard with the strongest evidence for preventing recurrent rheumatic fever. 1, 2, 3
• For high-risk patients with residual carditis, administration every 3 weeks may be necessary. 1
• This regimen has Class I, Level of Evidence B recommendation from the ACC/AHA. 1

Alternative Regimens for Penicillin Allergy

• Oral penicillin V: 250 mg twice daily 1, 3
• Sulfadiazine: 1 g orally once daily for patients >27 kg (60 lb); 0.5 g once daily for patients ≤27 kg 1, 3
• Macrolide or azalide antibiotics (for patients allergic to both penicillin and sulfadiazine), though these should not be used with cytochrome P450 3A inhibitors such as azole antifungals, HIV protease inhibitors, or certain SSRIs. 1

Duration of Prophylaxis

The duration is risk-stratified based on cardiac involvement: 1, 3

• Rheumatic fever WITH carditis AND residual valvular disease: 10 years after last attack OR until age 40 (whichever is longer); lifelong prophylaxis may be considered for high-risk patients with ongoing streptococcal exposure 1, 2, 3
• Rheumatic fever WITH carditis but NO residual heart disease: 10 years after last attack OR until age 21 (whichever is longer) 3
• Rheumatic fever WITHOUT carditis: 5 years after last attack OR until age 21 (whichever is longer) 3

Critical Consideration for Severe Valvular Disease

Recent evidence suggests that patients with severe valvular disease (severe mitral stenosis, aortic stenosis, aortic insufficiency) or reduced left ventricular systolic function may be at elevated risk of cardiovascular compromise following BPG injections. 4 For these high-risk patients, oral prophylaxis should be strongly considered as the risk of adverse reaction may outweigh theoretical benefit. 4

Prophylaxis After Valve Surgery

Secondary antibiotic prophylaxis must continue even after valve replacement surgery, following the same duration guidelines as non-surgical patients (minimum 10 years or until age 40 for severe RHD). 1, 3 This is a critical point—valve surgery does not eliminate the risk of recurrent acute rheumatic fever. 3

Medical Management of Cardiac Complications

Heart Failure Management

For patients with left ventricular systolic dysfunction, standard guideline-directed medical therapy should be implemented: 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 for advanced heart failure 1, 2
• Biventricular pacing when appropriate 1

Blood Pressure Management

• In patients with stenotic valve lesions, avoid abrupt lowering of blood pressure. 1, 2
• Maintain standard management of hypertension, diabetes, and hyperlipidemia. 1

Atrial Fibrillation and Anticoagulation

• Anticoagulation is indicated for stroke prevention in patients with atrial fibrillation and valvular heart disease. 1, 2

Infective Endocarditis Prophylaxis

Patients with rheumatic heart disease require antibiotic prophylaxis before dental procedures involving manipulation of gingival tissue, periapical region of teeth, or perforation of oral mucosa. 2

• For patients already receiving penicillin prophylaxis who need endocarditis prophylaxis, use an agent other than penicillin (such as clindamycin), as oral α-hemolytic streptococci likely have developed penicillin resistance. 3, 5
• Patients with prosthetic valves or prosthetic material used in valve repair require endocarditis prophylaxis for high-risk procedures. 3
• Optimal oral health maintenance is the most important component of preventing infective endocarditis. 1

Interventional Management

Indications for Valve Intervention

Percutaneous or surgical intervention is recommended for: 2

• Moderate-severe mitral stenosis with symptoms 2
• Asymptomatic patients with severe rheumatic mitral stenosis before pregnancy 2
• Valve replacement when percutaneous mitral balloon commissurotomy is contraindicated or unsuccessful 2

Monitoring and Follow-Up

Echocardiographic Surveillance

Regular echocardiographic monitoring intervals: 2

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

Vaccinations

• Influenza and pneumococcal vaccinations should follow standard recommendations. 1, 2

Special Considerations in Pregnancy

Women with moderate-severe rheumatic heart disease should be evaluated before pregnancy, with interventional therapy considered prior to conception. 2 During pregnancy, medical management includes beta-blockers, diuretics, and anticoagulation as needed. 2

Lifestyle and General Health

• Regular aerobic exercise improves cardiovascular fitness in most patients with asymptomatic valvular heart disease. 1
• Heavy isometric repetitive training should be avoided, but resistive training with small free weights or isolated muscle training is acceptable. 1
• Heart-healthy lifestyle factors (healthy diet, not smoking, maintaining normal body weight) apply equally to patients with valvular heart disease. 1

Critical Pitfalls to Avoid

• Never discontinue secondary prophylaxis prematurely—this is the most common and dangerous error. 2, 3
• Do not assume valve replacement eliminates the need for secondary prophylaxis—patients remain susceptible to group A streptococcus infection. 3
• Recognize pregnancy as a high-risk period requiring pre-conception evaluation and specialized management. 2
• Do not neglect regular echocardiographic follow-up, as disease progression can be insidious. 2
• Ensure adequate anticoagulation monitoring in patients with atrial fibrillation. 2
• Be aware of cardiovascular compromise risk with BPG injections in patients with severe valvular disease or reduced ventricular function. 4