What are the latest treatment guidelines for a young patient with Rheumatic Heart Disease (RHD) in a developing country?

Latest Treatment Guidelines for Rheumatic Heart Disease

The cornerstone of RHD treatment is long-term secondary antibiotic prophylaxis with benzathine penicillin G 1.2 million units intramuscularly every 4 weeks (or every 3 weeks in high-risk situations), combined with stage-based management according to the 2023 World Heart Federation guidelines and guideline-directed medical therapy for cardiac complications. 1, 2

Secondary Antibiotic Prophylaxis: The Foundation

Benzathine penicillin G remains the gold standard for preventing recurrent rheumatic fever and halting disease progression. 1, 2

• Administer 1.2 million units intramuscularly every 4 weeks as standard dosing 1, 2
• For high-risk patients (those with residual heart disease, high GAS exposure risk, or in endemic areas), shorten the interval to every 3 weeks to maintain more consistent protective penicillin levels 1, 2
• For penicillin-allergic patients, use oral penicillin V 250 mg twice daily as second-line, or sulfadiazine 1 gram orally once daily (0.5 gram if weight ≤27 kg) 1, 2
• Continue prophylaxis even after valve replacement surgery, as the risk of recurrent acute rheumatic fever persists 1, 2

Duration of Secondary Prophylaxis

The duration depends on disease severity and must be individualized based on specific criteria: 2

• Rheumatic fever with carditis and residual valvular disease: Continue for 10 years or until age 40 (whichever is longer) 2
• Rheumatic fever with carditis but no residual valvular disease: Continue for 10 years or until age 21 (whichever is longer) 2
• Rheumatic fever without carditis: Continue for 5 years or until age 21 (whichever is longer) 2
• Lifelong prophylaxis should be considered for patients at high risk of group A streptococcus exposure 2

Stage-Based Management According to 2023 WHF Guidelines

The 2023 World Heart Federation guidelines introduced a new stage-based classification system that reflects disease progression risk: 2

Stage A Disease (Low-Risk Early Disease)

• For individuals aged ≤20 years with stage A disease, consider secondary antibiotic prophylaxis after discussion with family and clinician, accounting for health-system factors and potential adverse effects 2, 3
• Mandatory follow-up echocardiography and longitudinal clinical evaluation must be established to monitor for disease progression 2, 3
• Continue SAP until follow-up echocardiogram is obtained 2

Stage B Disease (Moderate-to-High Risk)

• All individuals aged ≤20 years with stage B disease require secondary antibiotic prophylaxis due to moderate-to-high risk of progression 2, 3
• The number needed to treat is 13 to prevent one case of RHD progression in children aged 5-17 years with early-stage disease 3
• Regular echocardiographic surveillance at intervals determined by disease stage and clinical course 3

Stage C and D Disease (Advanced Disease)

• All patients with stage C or D disease require secondary antibiotic prophylaxis following local RHD management guidelines 2
• Valve intervention becomes necessary when patients develop symptomatic severe disease, severe valvular dysfunction with left ventricular systolic dysfunction, or refractory heart failure 1

Medical Management of Cardiac Complications

Apply standard guideline-directed medical therapy for left ventricular systolic dysfunction and heart failure complications: 1, 2

• Diuretics for volume overload and congestion 1, 2
• ACE inhibitors or ARBs for afterload reduction and ventricular remodeling 1, 2
• Beta-blockers for heart rate control and neurohormonal blockade 1, 2
• Aldosterone antagonists for additional neurohormonal blockade in advanced heart failure 1, 2
• Sacubitril/valsartan may be considered in appropriate candidates 2

Critical Hemodynamic Consideration

In patients with stenotic valve lesions (mitral stenosis or aortic stenosis), avoid abrupt lowering of blood pressure to prevent hemodynamic collapse 1, 2

Management of Acute Rheumatic Carditis

If clinical evidence of acute carditis exists alongside chronic RHD, initiate anti-inflammatory therapy: 3

• Prednisone 1-2 mg/kg/day for 1-2 weeks is recommended for myocarditis associated with acute rheumatic fever 3
• Continue secondary antibiotic prophylaxis alongside anti-inflammatory treatment 3

Anticoagulation for Atrial Fibrillation

Warfarin remains the only oral anticoagulant licensed for use in valvular heart disease, requiring regular therapeutic monitoring 2

• Point-of-care portable monitoring of anticoagulation status is feasible and improves clinical care, particularly important in resource-limited settings 2
• Atrial fibrillation is common in RHD due to left atrial enlargement from mitral valve disease 4, 5

Infective Endocarditis Prophylaxis: Clarifying the Misconception

Current guidelines do NOT recommend routine endocarditis prophylaxis for rheumatic heart disease alone 1, 2

Antibiotic prophylaxis before dental procedures is reasonable ONLY for patients with: 2

• Prosthetic cardiac valves (including transcatheter-implanted prostheses)
• Prosthetic material used for cardiac valve repair
• Previous infective endocarditis
• Unrepaired cyanotic congenital heart disease

The maintenance of optimal oral health remains the most important component in preventing infective endocarditis 2

Additional Preventive Measures

• Influenza and pneumococcal vaccinations should follow standard recommendations 1, 2
• Regular aerobic exercise is encouraged to improve cardiovascular fitness in patients with asymptomatic valvular disease 1

Diagnostic Confirmation with Echocardiography

The 2023 WHF guidelines provide updated echocardiographic criteria for definitive RHD diagnosis: 2

Pathological Mitral Regurgitation (All Criteria Must Be Met)

• Observed in at least two views 2
• Jet length ≥1.5 cm in patients weighing <30 kg or aged <10 years 2
• Jet length ≥2.0 cm in patients weighing ≥30 kg or aged ≥10 years 2, 3
• Peak velocity ≥3.0 m/s 3
• Pan-systolic jet 3

Pathological Aortic Regurgitation

• Jet length ≥1.0 cm 3
• Peak velocity ≥3.0 m/s in early diastole 3
• Pan-diastolic jet 3

Mitral Stenosis

• Restricted leaflet motion with thickening and calcification 3, 5
• Mean peak gradient ≥4.0 mmHg 3

Critical Pitfalls to Avoid

Before initiating long-term prophylaxis, administer a full therapeutic course of penicillin to eradicate any residual group A streptococcus 1

Secondary prophylaxis must continue even after valve replacement surgery, as the risk of recurrent acute rheumatic fever from group A streptococcus infection persists 1, 2

Macrolide antibiotics should not be used in persons taking medications that inhibit cytochrome P450 3A, such as azole antifungals, HIV protease inhibitors, and some selective serotonin reuptake inhibitors 2

In patients with stenotic lesions, abrupt blood pressure lowering can precipitate hemodynamic collapse 1

Surgical and Interventional Considerations

Valve surgery and percutaneous valvuloplasty are associated with substantially lower mortality (HR 0.23 and 0.24 respectively) in appropriate candidates 6

Percutaneous balloon mitral valvuloplasty (PBMV) is the treatment of choice for severe mitral stenosis with suitable valve anatomy and without significant mitral regurgitation 7

However, access to surgical and interventional care remains severely limited in endemic regions, with only 18 procedures per million population performed in Africa compared to 1,222 per million in the USA 2

Register-Based Comprehensive Control Programs

The World Heart Federation advocates for comprehensive register-based control programs that include: 2

• Conducting surveys to determine disease burden
• Identifying cases of known or suspected RF and RHD
• Maintaining centralized registers of all known cases
• Standardizing guidelines for monitoring and improving delivery of secondary prophylaxis
• Training key health workers and maintaining a skilled workforce
• Educating patients and their families

Prognosis and Mortality Data

Mortality in RHD remains unacceptably high at 4.7% per patient-year, with most deaths due to vascular causes (67.5%), mainly heart failure or sudden cardiac death 6

Markers of severe valve disease associated with increased mortality include: 6

• Congestive heart failure (HR 1.58)
• Pulmonary hypertension (HR 1.52)
• Atrial fibrillation (HR 1.30)

Without intervention, the disease leads to premature death, with mean age of death <25 years in some studies 2, 4