Rheumatic Heart Disease¶

Learning Objectives¶

Core Knowledge & Clinical Reasoning¶

[ ] Apply 2023 WHF echocardiographic criteria to diagnose definite vs borderline RHD
[ ] Differentiate morphological mitral valve features and pathological regurgitation criteria
[ ] Determine secondary prophylaxis duration by category (5 years to lifelong based on carditis/residual disease)
[ ] Recognize that subclinical carditis on echo counts as MAJOR Jones criterion

Management Decisions¶

[ ] Prescribe appropriate secondary prophylaxis (benzathine PCN G every 3-4 weeks preferred)
[ ] Formulate primary prevention strategy for GAS pharyngitis
[ ] Determine when valve surgery is indicated for severe RHD

Communication & Counseling¶

[ ] Counsel families on importance of prophylaxis adherence and consequences of recurrence
[ ] Explain lifelong surveillance requirements for patients with residual heart disease
[ ] Address socioeconomic barriers to prophylaxis access in high-prevalence settings

Systems-Based Practice¶

[ ] Implement school-based or community screening programs in endemic areas
[ ] Ensure systematic prophylaxis tracking to prevent missed doses
[ ] Coordinate transition to adult care with ongoing prophylaxis requirements

Key Guidelines¶

2020 AHA Scientific Statement: Diagnosis and Management of Rheumatic Heart Disease Circulation. 2020;142:e337-e357

2023 World Heart Federation Echo Criteria for RHD Nat Rev Cardiol. 2024;21:250-263

2023 ASE Recommendations for Echo in RHD J Am Soc Echocardiogr. 2023;36:3-28

2024 WHO Guideline on Prevention and Diagnosis of RF/RHD

2023 WHF Echocardiographic Criteria¶

Definite RHD¶

Pathological MR + morphological MV changes, OR
MS with mean gradient ≥4 mmHg

Borderline RHD¶

≥2 morphological MV features, OR
Pathological AR in age <20 years (without MV changes)

Morphological MV Features¶

Feature	Threshold
Anterior MV leaflet thickening	≥3 mm (age-specific)
Chordal thickening	Present
Restricted leaflet motion	Present
Excessive leaflet tip motion	Present

Pathological MR Criteria¶

Jet seen in ≥2 views
Jet length ≥2 cm in at least 1 view
Peak velocity >3 m/s
Pansystolic jet in at least 1 envelope

Pathological AR Criteria¶

Jet seen in ≥2 views
Jet length ≥1 cm in at least 1 view
Peak velocity >3 m/s
Pandiastolic jet

Secondary Prophylaxis¶

Duration by Category¶

Category	Duration
ARF without carditis	5 years or until age 21 (whichever longer)
ARF with carditis, no residual disease	10 years or until age 21 (whichever longer)
ARF with residual heart disease	10 years or until age 40 (whichever longer)
Severe RHD or after valve surgery	Lifelong

Regimen Options¶

Agent	Dose	Route	Frequency
Benzathine PCN G (preferred)	1.2 million units	IM	Every 3-4 weeks
Penicillin V	250 mg	PO	BID
Erythromycin (PCN allergy)	250 mg	PO	BID

Primary Prevention¶

Treat GAS pharyngitis: - Penicillin V 250 mg PO BID-TID x 10 days, OR - Benzathine PCN G 1.2 million units IM x 1 dose - Amoxicillin 50 mg/kg/day (max 1g) x 10 days

Screening Programs¶

WHO Recommendations¶

Echo screening in high-prevalence settings
School-based programs effective
Early detection before symptoms

Screening Benefits¶

Earlier prophylaxis initiation
Prevention of ARF recurrence
Reduced valve disease progression

Board Pearls¶

Pearl: Secondary prophylaxis: Benzathine PCN G every 3-4 weeks

Duration depends on carditis presence and residual disease

Pearl: Lifelong prophylaxis after valve surgery

Also for severe RHD

Pearl: Subclinical carditis on echo counts as MAJOR criterion for ARF

2015 Jones Criteria update

Self-Assessment¶

Q1: A 10-year-old had ARF 2 years ago with documented carditis. Follow-up echo shows no residual valve disease. How long should secondary prophylaxis continue?

Answer

**Answer**: 10 years or until age 21, whichever is longer (so until age 21) **Rationale**: ARF with carditis but no residual disease requires 10 years of prophylaxis or until age 21, whichever is longer. Since the child is now 10, prophylaxis would continue until age 20 (10 years) or 21, meaning age 21.

Q2: What is the preferred agent and regimen for secondary RHD prophylaxis?