Rheumatic Heart Disease

• Presented by
• Institution
• Date: March 3, 2025
 Rheumatic Heart Disease

– Acute Rheumatic Fever (ARF): A post-infectious autoimmune condition

triggered by Group A Streptococcus (GAS) pharyngitis.
– Rheumatic Heart Disease (RHD): The chronic sequela of ARF, characterized by
progressive valvular damage.
– Up to 60% of patients with ARF progress to RHD (i.e. Approximately 50 - 60 %
of those with evidence of carditis develop organic valvular damage).
– The valvular lesions begin as small verrucae composed of fibrin and blood cells
along the borders of one or more of the heart valves.
– With repeated attacks of rheumatic fever, new verrucae form near the previous
ones, and the mural endocardium and chordae tendineae become involved
– Up to 75% of patients with documented recurrences of RF have some form of
valvular disease after 4 to 5 years of follow-up
– RHD is the most common cause of heart disease in children in developing
countries and is a major cause of mortality and morbidity in adults as well 
RHD, peaks between 25 and 40 years.
 Rheumatic Heart Disease

– RHD more commonly affects females, sometimes up to twice as frequently as

males.
– RHD remains a major cause of cardiovascular disease in developing nations
– In Africa valvular disease is encountered in the young, not infrequently in
children of school-going age or young females of child-bearing potential, and
with a course that is much more rapid.
– RHD accounts for 12 - 65% of hospital admissions related to cardiovascular
disease and 2 - 10% of hospital discharges in some developing countries.
– In Ethiopia, RHD Accounts for 50 % of all Cardiovascular disease admissions to
hospital and common in females
Rheumatic Heart Disease
 Clinical Features

– People with RHD are often asymptomatic for many years before their valvular
disease progresses to cause cardiac failure.
– While chronic RHD occurs only as a sequalae of ARF, the majority of patients with
RHD lack a history of past ARF, suggesting that the diagnosis of ARF is frequently
missed with the initial or recurrent insults being subclinical or not detected.
– RHD generally presents as valve disease, which may or may not be detected by a
murmur.
– The frequency of symptoms and signs of HF (eg, dyspnea and evidence of
pulmonary edema) and of embolic events (eg, stroke) among patients with RHD
varies depending upon the stage of valve disease at diagnosis.
– In endemic areas presentation with advanced disease is common
– Complications of RHD may happen such as HF, Atrial fibrillation, IE, Pulmonary
hypertension and Cardio-embolic stroke (less common)
– Multivalvular disease (varying combinations of stenotic or regurgitant lesions
involving the mitral and aortic valves) is the predominant abnormality in older
adults
 Clinical Features

– The Mitral Valve is involved (MR with or Without MS) in nearly all cases of RHD,
including those with other affected valves.
– The aortic valve is involved (AR with or Without AS) in approximately 20 to 30 %
of cases.
– The tricuspid valve is commonly affected, but tricuspid valve disease is
frequently subclinical until surgery is required.
– RHD can cause organic TR and/or tricuspid stenosis.
– Rheumatic tricuspid valve disease is almost invariably associated with Mitral
Valve disease ✓ Pulmonic valve involvement is rare.
– So, the Clinically detectable valvular findings from RHD patients in our setup are
MR and TR with/without AR
– The diagnosis of RHD is generally confirmed by transthoracic echocardiography,
which enables assessment of valve morphology and severity of valve dysfunction
(regurgitation and/or stenosis). ✓ Echocardiography is also used to assess left
and right ventricular size and function and may enable estimation of pulmonary
artery systolic pressure.
 Epidemiology

– Global Impact: 40.5 million cases (2019 estimate), 300,000-500,000 new ARF
cases annually.
– High-Risk Regions:
• Sub-Saharan Africa: 5.7 cases/1,000 children.
• South Asia: 3-5 million cases.
• Indigenous Populations: e.g., 1 in 50 Aboriginal Australian children.
– Social Determinants: Overcrowding (↑GAS transmission), poor sanitation,
limited antibiotic access.
– Trends: Near-eradicated in high-income countries (e.g., USA: <0.05/1,000);
persistent in low-resource settings.
– Research Insight: Climate change may worsen RHD by increasing overcrowding
in vulnerable areas.
 Epidemiology – Risk Factors

– Socioeconomic: Poverty, overcrowding, poor sanitation.

– Biological: Genetic predisposition (e.g., HLA-DR7 association).
– Environmental: Limited access to healthcare, untreated strep infections.
– Gender: Slight female predominance in some studies.
 Epidemiology

– Prevalence: Estimated 33.4 million cases worldwide; 80% in low- and middle-
income countries.
– Incidence: Approximately 471,000 new ARF cases annually, leading to 233,000
deaths from RHD.
– High-Risk Areas: Sub-Saharan Africa, South Asia, Indigenous populations in
Australia.
– Age Group: Predominantly affects children aged 5–15 years.

Prevalence (%)
8
6
4
2
0
Africa Asia Pacific Europe Americas
 Diagnosis

– Acute Rheumatic Fever (ARF):

• Diagnosed using Jones Criteria (2 major or 1 major + 2 minor criteria).
• Evidence of GAS infection: Positive throat culture, elevated ASO titers.
• Echocardiography: Detects subclinical carditis.
– Rheumatic Heart Disease (RHD):
• Echocardiography: Gold standard for detecting valve damage.
• Clinical signs: Murmurs, heart failure symptoms.
• ECG: Prolonged PR interval, atrial fibrillation.
 Jones Criteria & 2015 Revision

Jones Criteria: is used to diagnose Acute Rheumatic Fever.

A patient must meet "2 major or 1 major + 2 minor criteria" with evidence of recent
GAS infection.
Major Criteria:
- Carditis: Heart inflammation (murmurs, pericarditis).
- Polyarthritis: Migratory joint pain/swelling.
- Sydenham’s chorea: Involuntary movements.
- Erythema marginatum: Pink, non-itchy rash.
- Subcutaneous nodules: Painless lumps.

Minor Criteria:
- Fever, arthralgia, elevated inflammatory markers (CRP, ESR), prolonged PR interval on
ECG.

2015 Revision:
- Subclinical Carditis now a major criterion in high-risk populations.
- High-risk populations: Those in endemic areas with limited healthcare access.
 Differential Diagnosis

• Conditions Mimicking ARF:

- Infective Endocarditis: Fever, murmurs, positive blood cultures.
- Lyme Disease: Arthritis, neurological symptoms, history of tick bite.
- Juvenile Idiopathic Arthritis: Chronic joint inflammation without carditis.
- Systemic Lupus Erythematosus: Multisystem autoimmune disease (positive ANA,
anti-dsDNA).
- Viral Myocarditis: Heart inflammation after a viral illness.
• Conditions Mimicking RHD:
- Congenital Heart Disease: Valve abnormalities from birth.
- Degenerative Valve Disease: Age-related valve degeneration.
- Infective Endocarditis: Valve damage due to infection.
Differential Diagnosis
 Investigation

Laboratory Tests:
• Throat culture/rapid antigen test for GAS.
• ASO and anti-DNase B titers (rising levels confirm recent infection).
• Inflammatory markers: CRP (>10 mg/L), ESR (>30 mm/h).

Imaging:
• Echocardiography: Gold standard—detects valvular regurgitation/stenosis.
• Chest X-ray: Cardiomegaly or pulmonary edema in severe cases.
• ECG: Prolonged PR interval (1st-degree AV block) in carditis.
 Management
Acute Phase:
• Eradicate GAS: Penicillin (single IM benzathine or 10-day oral course).
• Reduce inflammation: Aspirin (high-dose for arthritis), corticosteroids (severe
carditis)
Supportive Care:
• Bed rest, heart failure management (diuretics, ACE inhibitors).
Chronic Phase:
• Secondary prophylaxis: IM benzathine penicillin every 3-4 weeks for 5-10+ years.
• Regular echocardiography for valve progression monitoring.
 Rheumatic Valvular Heart Disease

 Valvular Heart Disease (VHD) encompasses stenosis or regurgitation of the mitral,

aortic, tricuspid, or pulmonary valves. CRMVHD, secondary to rheumatic fever (RF),
is the leading cause globally, affecting 40 million, particularly in developing
countries.
 Degenerative VHD (e.g., aortic stenosis) rises with age, impacting 12–13% of adults
over 75. Infective endocarditis (IE) is an emerging cause of acute regurgitation.
 Prevalence increases with age, reflecting diverse etiologies—rheumatic,
degenerative, congenital, and infectious—driving significant morbidity (13 million
DALYs annually).
 CRMVHD peaks in children/young adults (5–15 years) in low-income settings
(incidence 150/100,000), causing 80% of VHD cases there. Degenerative VHD, like
aortic stenosis, affects 12–13% of those over 75 in high-income countries.
 RF remains the primary driver in developing nations, while IE rises globally due to
invasive procedures.
 Aging increases VHD prevalence universally, with left-sided valves (mitral, aortic)
most impacted, necessitating targeted screening and prevention.
Rheumatic Valvular Heart Disease
 Pathophysiology
 CRMVHD results from RF, an autoimmune sequela of Group A Streptococcus
pharyngitis. Molecular mimicry between GAS M-protein and cardiac antigens
triggers inflammation, forming Aschoff bodies. Chronic fibrosis affects multiple
valves—mitral (70–80%), aortic (20–30%), tricuspid (10%)—causing stenosis or
regurgitation. This multivalvular pathology distinguishes CRMVHD as a leading VHD
etiology in LMICs.

 Degenerative VHD arises from calcific changes (e.g., aortic stenosis) in the elderly,
driven by hypertension and dyslipidemia. IE causes acute valve destruction via
bacterial vegetations. Congenital anomalies (e.g., bicuspid aortic valve) and
connective tissue disorders (e.g., Marfan syndrome) contribute to chronic lesions.
Each etiology dictates lesion type and progression, necessitating etiology-specific
management.
 Aortic Regurgitation (AR)

• AR (aortic insufficiency) results from inadequate aortic valve closure, causing

diastolic backflow into the left ventricle (LV). Acute AR (e.g., IE, dissection) is a
surgical emergency with high mortality. Chronic AR progresses slowly from RHD,
congenital defects, or aortic root dilation. It affects 0.5–1% of adults, rising with
age, and imposes volume overload, leading to LV dilation and heart failure if
untreated.
Causes
• Acute AR arises from IE (vegetations), aortic dissection (root disruption), or trauma
(iatrogenic, e.g., failed valve surgery). Chronic AR includes primary valve disease—
RHD (leaflet scarring), IE, bicuspid valve, SLE, syphilis, ankylosing spondylitis,
myxomatous prolapse—and aortic root dilation from dissection, severe
hypertension, Marfan’s, cystic medial degeneration, aortitis, or idiopathic causes.
Root widening separates leaflets, exacerbating regurgitation.
 Aortic Regurgitation (AR)

Clinical Features
• Chronic AR symptoms include dyspnea on exertion, paroxysmal nocturnal dyspnea
(PND), orthopnea, palpitations (worse lying down), angina, and heartbeat
awareness. Acute AR presents with cyanosis and shock. Exam reveals widened
pulse pressure (e.g., 160/50 mmHg), displaced PMI, AR murmur (diastolic, high-
pitched), and peripheral signs: deMusset’s (head bob), Traube’s (pistol shot pulse),
Duroziez’s (femoral bruit), Quincke’s (capillary pulsations), Hill’s (popliteal > brachial
pressure).
Diagnosis
• Diagnosis uses CXR (enlarged silhouette, dilated aorta), ECG (LVH), and
echocardiography—assessing LV size, function, aortic root dilation, and flow
reversal. Acute AR shows early mitral valve closure. Serial monitoring: mild AR
(echo every 2–3 years), moderate (1–2 years), severe (yearly). Doppler quantifies
regurgitation severity (e.g., jet width >65% = severe). Accurate diagnosis guides
timing of intervention.
 Aortic Regurgitation (AR)

Treatment
• Diagnosis uses CXR (enlarged silhouette, dilated aorta), ECG (LVH), and
echocardiography—assessing LV size, function, aortic root dilation, and flow
reversal. Acute AR shows early mitral valve closure. Serial monitoring: mild AR
(echo every 2–3 years), moderate (1–2 years), severe (yearly). Doppler
quantifies regurgitation severity (e.g., jet width >65% = severe). Accurate
diagnosis guides timing of intervention.
 Tricuspid Regurgitation (TR)

• TR results from inadequate tricuspid valve closure, often secondary (>80%) to right
ventricular (RV) dilatation from pulmonary hypertension or left-sided VHD. Primary
TR (e.g., RHD, IE) is less common. Prevalence rises with age and RV pathology,
causing right heart failure if severe. TR’s functional nature complicates
management, often requiring left-sided correction first.
Causes
• Primary TR includes RHD (leaflet scarring), IE (vegetations), papillary muscle injury
(post-MI), myxomatous prolapse, trauma, radiation, carcinoid, endomyocardial
fibrosis, and congenital Ebstein’s anomaly. Secondary TR stems from RV/tricuspid
annular dilatation due to MI, cardiomyopathy, AF, pulmonary hypertension, or left-
sided VHD (e.g., MS). Functional TR dominates clinical practice, reflecting RV
pressure/volume overload. Acute vs. Chronic AR Causes
 Tricuspid Regurgitation (TR)

Clinical Features
• Mild/moderate TR is asymptomatic unless RV failure develops. Severe TR causes
fatigue, dyspnea, cervical pulsations, abdominal bloating, anorexia, weight loss,
and leg edema. Signs include distended neck veins, hepatomegaly with systolic
pulsations, ascites, pleural effusions, hepatojugular reflux, left parasternal heave,
and a holosystolic murmur (intensified on inspiration—Carvallo’s sign). AF is
common in chronic TR.

Diagnosis
• ECG shows RVH, RA enlargement, AF, or bizarre RBBB in Ebstein’s. CXR reveals
RA/RV enlargement. Transthoracic echo (TTE) confirms TR, assessing severity (jet
area, vena contracta), RA/RV overload, and leaflet pathology (prolapse, scarring).
Severe TR reduces cardiac output (CO). Diagnosis guides therapy by distinguishing
primary vs. secondary causes.
 Tricuspid Regurgitation (TR)

TR – Treatment
• Diuretics (furosemide) and aldosterone antagonists manage severe TR with right
heart failure, addressing hepatic congestion. Left ventricular failure requires beta-
blockers/ARBs. Correcting pulmonary hypertension (e.g., from MS) improves
functional TR. Surgery (tricuspid repair/replacement) is indicated for severe TR with
left-sided surgery, moderate TR with annular dilation (>40 mm), or unresponsive
primary TR with RV dysfunction.
 Mitral Regurgitation (MR)

• MR, due to inadequate mitral valve closure, can be acute (high mortality) or
chronic. CRMVHD is a leading chronic cause, affecting 1–2% globally. Acute MR
(e.g., IE, papillary rupture) demands urgent intervention, while chronic MR
progresses slowly, imposing LV volume overload. Severity drives symptoms and
outcomes, with RHD often multivalvular.

Causes
• Acute MR results from IE (S. aureus), papillary muscle rupture (post-MI), chordal
rupture, or trauma. Chronic primary MR includes RHD, IE, mitral valve prolapse
(MVP), trauma, and congenital defects. Secondary MR arises from LV dilatation
(IHD, DCMP, HCMP, chronic AF) or mitral annular calcification (mixed
primary/secondary). RHD dominates in LMICs.
 Mitral Regurgitation (MR)

Clinical Features
• Symptoms include dyspnea on exertion, PND, orthopnea, palpitations, and late
pulmonary edema. Signs feature an active precordium, displaced PMI, apical
systolic thrill, S3 gallop, and a holosystolic murmur radiating to the axilla.
Severity correlates with LV failure risk, with acute MR presenting as
cardiogenic shock. Chronic MR may be asymptomatic until advanced.

Diagnosis
• CXR shows cardiomegaly, dilated LV, and pulmonary edema. ECG reveals AF or
LVH. Echocardiography (Doppler, color flow) confirms MR, assessing LA/LV size,
LV function, and severity (e.g., regurgitant fraction >50% = severe).
Monitoring: mild MR (yearly clinical), moderate (echo yearly), severe (echo 6–
12 months). Etiology guides therapy.
 Mitral Regurgitation (MR)

Treatment
• Asymptomatic MR requires follow-up; hypertension is treated with
enalapril/amlodipine. Symptomatic MR uses Lasix (40 mg BID) and enalapril
(2.5–20 mg/day). LVEF <40% follows HFrEF protocols. AF and anticoagulation
mirror MS management. Surgery (repair/replacement) is indicated for
symptomatic severe MR, LVEF <60%, LVESD >40 mm, new AF, or pulmonary
hypertension. RHD prophylaxis applies if rheumatic.
 Mitral Stenosis (MS)

• MS, more common in women, narrows the mitral valve (normal 4–6 cm²),
becoming symptomatic at <1.5 cm². CRMVHD is the primary cause, affecting
0.1–0.2% globally, with severe cases (<1 cm²) driving heart failure. Left atrial
pressure rises, causing pulmonary congestion, making MS a critical VHD
subtype requiring timely intervention.

Causes
• MS is predominantly rheumatic (CRMVHD), with leaflet thickening and
commissural fusion from repeated RF. Rare causes include congenital MS,
mitral annular calcification, radiation, or metabolic disorders (e.g., Fabry’s).
Severity is classified: mild (1.5–2 cm²), moderate (1–1.5 cm²), severe (<1 cm²).
RHD’s multivalvular impact often coexists with MR or AR.
 Mitral Stenosis (MS)

Clinical Features
• Asymptomatic until MVA <1.5 cm², MS then causes dyspnea, orthopnea, fatigue,
and palpitations. Severe cases add hemoptysis, pulmonary edema, and right heart
failure (edema, hepatomegaly). Exam reveals a diastolic rumble, opening snap,
loud P2 (pulmonary hypertension), and AF (40–50%). Symptoms worsen with
pregnancy or exertion.

Diagnosis
• CXR shows left atrial enlargement, pulmonary congestion. ECG indicates LA
enlargement (P mitrale), AF, or RVH. Echocardiography assesses MVA (e.g., <1 cm²
= severe), gradient (>10 mmHg = severe), and pulmonary pressures. Serial
monitoring mirrors MR: mild (yearly clinical), moderate (yearly echo), severe (6–
12 months). Doppler quantifies obstruction.
 Mitral Stenosis (MS)

Treatment
• Asymptomatic MS needs monitoring; symptomatic cases use diuretics
(furosemide) and rate control (beta-blockers) for AF. Anticoagulation (warfarin,
INR 2–3) prevents thromboembolism in AF or LA thrombus. Severe MS (MVA
<1 cm²) with symptoms or pulmonary hypertension requires balloon
valvuloplasty or surgery (replacement if calcified). RHD prophylaxis prevents
recurrence.
 Surgical Intervention

Indications:
Severe valvular dysfunction or heart failure.
Options:
• Valve repair (e.g., commissurotomy for mitral stenosis).
• Valve replacement (mechanical or bioprosthetic).
Challenges:
• High cost, lifelong anticoagulation needs (mechanical valves), limited access in
endemic areas.
Outcomes:
• Significant improvement in quality of life but requires lifelong management.
 Prevention

Primary Prevention:
Early treatment of GAS pharyngitis with penicillin (IM or oral) within 9 days of
symptom onset.
Secondary Prevention:
Long-term penicillin prophylaxis (IM every 3-4 weeks) tailored to severity (e.g., until
age 21 or 10 years post-ARF).
Tertiary Prevention:
Managing RHD complications (e.g., surgery, heart failure medications).
Public Health:
Reduce overcrowding, improve sanitation, train healthcare workers in endemic areas.
 Conclusion

RHD remains a preventable but persistent global challenge.

Early intervention and public health strategies are key.
Question ?
Thank You!
RHD Prevalence in High-Risk
Regions
Treatment Effectiveness for RHD
RHD Disease Progression Flowchart
Echocardiography Findings in RHD
Heart Valve Damage in RHD
Global Burden of RHD by Region