A common heart condition that is important especially to the paediatric population

1. RHEUMATIC HEART DISEASE
PRESENTER:DR.KIMETO

2. OUTLINE
• Introduction
• Epidemiology
• Aetiology
• Pathogenesis
• Clinical manifestations
• Management
• Complications
• Prevention

4. Introduction
• Acute rheumatic fever (ARF) is a delayed, nonsuppurative sequela of a pharyngeal infection with
the group A streptococcus (GAS).
• Following the initial pharyngitis, a latent period of two to three weeks occurs before the first signs
or symptoms of Acute Rheumatic Fever appear.
• In developing areas of the world, acute rheumatic fever and rheumatic heart disease are estimated
to affect nearly 20 million people and are the leading causes of cardiovascular death during the first
5 decades of life.
• Worldwide, there are 470,000 new cases of rheumatic fever and 233,000 deaths attributable to
rheumatic fever or rheumatic heart disease each year; most occur in developing countries and
among indigenous groups.
• The mean incidence of Acute Rheumatic Fever is 19 per 100,000.

5. Epidemiology
• Age 5 to 15 years are more susceptible.(average age of 10years)
• M=F although the prognosis is though to be worse in females
• Common in developing countries.
• Incidence more common in cold season
• Environmental factors :overcrowding, poor sanitation, poverty.

6. Aetiology
• Acute rheumatic fever is a systemic disease of childhood often that follows a group A beta
hemolytic streptococcal infection.
• Rheumatic fever is thought to result from an inflammatory autoimmune response.
• Rheumatic fever only develops in children and adolescents following group A beta-hemolytic
streptococcal pharyngitis, and only streptococcal infections of the pharynx initiate or reactivate
rheumatic fever.
• The proposed pathophysiology for development of rheumatic heart disease is as follows:
 Cross-reactive antibodies bind to cardiac tissue facilitating infiltration of streptococcal-primed
CD4+ T cells
 trigger an autoimmune reaction releasing inflammatory cytokines (including TNF-alpha and IFN-
gamma).
 Because few IL-4–producing cells are present in valvular tissue, inflammation persists, leading to
valvular lesions.

7. Pathogenesis
• Rheumatic fever develops in some children and adolescents following pharyngitis with group A
beta-hemolytic Streptococcus (ie, Streptococcus pyogenes).
• The organisms attach to the epithelial cells of the upper respiratory tract and produce a battery of
enzymes allowing them to damage and invade human tissues.
• After an incubation period of 2-4 days, the invading organisms elicit an acute inflammatory
response with 3-5 days of sore throat, fever, malaise, headache, and an elevated leukocyte count.
• In 0.3-3% of cases, infection leads to rheumatic fever several weeks after the sore throat has
resolved.
• Group A streptococci may be subserotyped by surface proteins on the cell wall of the organism.
The presence of the M protein is the most important virulence factor for group A streptococcal
infection in humans.
• Anti-M antibodies against the streptococcal infection may cross-react with components of heart
tissue (ie, sarcolemmal membranes, valve glycoproteins).

8. Pathogenesis
• Acute rheumatic heart disease often produces a pancarditis characterized by endocarditis,
myocarditis, and pericarditis.
• Endocarditis is manifested as valve insufficiency. The mitral valve is most commonly and severely
affected (65-70% of patients), and the aortic valve is second in frequency (25%).
• The tricuspid valve is deformed in only 10% of patients and is almost always associated with
mitral and aortic lesions. The pulmonary valve is rarely affected.
• Severe valve insufficiency during the acute phase may result in congestive heart failure and even
death (1% of patients).
• Chronic manifestations due to residual and progressive valve deformity occur in 9-39% of adults.
• Fusion occurs at the level of the valve commissures, cusps, chordal attachments, or any
combination of these resulting in stenosis or a combination of stenosis and insufficiency.
• Associated atrial fibrillation or left atrial thrombus formation from chronic mitral valve
involvement and atrial enlargement may be observed.

9. Clinical Presentation
• Previous Hx of throat infection or rheumatic fever.
• The Jones criteria require the presence of 2 major or 1 major and 2 minor criteria along with
evidence for recent streptococcal infection for the diagnosis of rheumatic fever.
• The major diagnostic criteria include: carditis, polyarthritis, chorea, subcutaneous nodules, and
erythema marginatum.
• The minor diagnostic criteria include: fever, arthralgia, prolonged PR interval on ECG, elevated
acute phase reactants (increased erythrocyte sedimentation rate [ESR]), presence of C-reactive
protein, and leukocytosis.
• After a diagnosis of rheumatic fever is made, symptoms consistent with heart failure eg difficulty
breathing, exercise intolerance, tachycardia, may be indications of carditis and rheumatic heart
disease.

10. Investigations
• Throat culture
• Antistreptococcal antibody test(ASOT).
• Acute phase reactants-C reactive protein and ESR.
• Full Haemogram
• ECG –Prolonged PR interval,atrial flutter,atrial fibrillation.
• Imaging studies:
• CXR
• ECHO
• Diagnostic catheterization-balloon valvuloplasty.
• CT Scan Brain

11. Management
• supplemental oxygen, bed rest, and sodium and fluid restriction
• Diuretics which include furosemide and spironolactone.
• Digoxin (only after checking electrolytes and correcting hypokalemia).
• Afterload reduction ie using ACE inhibitor eg captopril
• Steroid therapy eg prednisone
• Junior Aspirin .
• Surgical intervention:
balloon mitral valvuloplasty
Open heart surgery-MV replacement

12. Complications
• Valve deformities, thromboembolism, cardiac hemolytic anemia, and atrial arrhythmias are the
most common cardiac manifestations of chronic rheumatic heart disease.
• Mitral stenosis occurs in 25% of patients with chronic rheumatic heart disease and in association
with mitral insufficiency in another 40%.
• Aortic stenosis from chronic rheumatic heart disease is typically associated with aortic
insufficiency.
• Thromboembolism occurs as a complication of mitral stenosis. It is more likely to occur when the
left atrium is dilated, cardiac output is decreased, and the patient is in atrial fibrillation
• Cardiac hemolytic anemia is related to disruption of the RBCs by a deformed valve. Increased
destruction and replacement of platelets also may occur.
• Atrial arrhythmias are typically related to a chronically enlarged left atrium (from a mitral valve
abnormality).

13. Mitral stenosis
• Mitral disease begins with the formation of tiny nodules located along the coapting portions of the
valve leaflets.
• The leaflets thicken with eventual deposition of fibrin on the cusps and loss of normal valve
morphology
• Disease progression results in a number of pathologic changes affecting the mitral valve apparatus,
which are diagnostic for rheumatic valve disease:
Fusion of the leaflet commissures
Thickening, fusion and shortening of the chordae tendineae
• In addition, there may superimposed thickening, fibrosis, and calcification of the leaflet cusps
• The net effect is a stenotic mitral valve with a symmetric, central oval-shaped orifice and a classic
pattern of "doming" of the leaflets in diastole due to fusion of the leaflet tips at the commissures

14. Mitral Stenosis
• The primary hemodynamic consequence of MS is :
 a pressure gradient between the LA and LV in diastole.
 elevated left atrial pressure is reflected backward, causing an ↑ in pulmonary venous, capillary,
and arterial pressures and resistance.
This leads to pulmonary hyperetension.
 With mild to moderate MS, these abnormalities are often only apparent with exercise or other
conditions that increase heart rate; they eventually are seen at rest as the severity of the stenosis
increases.
Thromboembolism occurs as a complication of mitral stenosis. It is more likely to occur when the
left atrium is dilated, cardiac output is decreased, and the patient is in atrial fibrillation

15. Mitral Stenosis-management
• Anti hypertensives-diuretics,digoxin,ACE inhibitors
• Patients who have thrombus formation -Steroid therapy eg prednisone or NSAIDs eg Junior Aspirin can
be used.
• Anticoagulants eg warfarin
• Mitral valvotomy indicated in:
 symptomatic NYHA Functional Class II–IV patients with isolated MS
 Mitral valve area is <1.5 cm2 .
• Mitral valvotomy can be carried out by two techniques:
 PMBV percutaneous mitral balloon valvotomy
 surgical valvotomy.
• Mitral Valve Replacement

16. Mitral Regurgitation
• The resistance to LV emptying (LV afterload) is reduced.
• The LV is decompressed into the LA during ejection, and with the reduction in LV size during
systole, there is a rapid decline in LV tension.
• This increase in LV volume is accompanied by a reduced forward CO
• The regurgitant volume varies directly with the LV systolic pressure and the size of the regurgitant
orifice; which is influenced by the extent of LV and mitral annular dilation.
• A modest reduction in ejection fraction (EF)ie <60% reflects significant dysfunction.

17. Mitral Regurgitation-Management
• Urgent stabilization.
• Antihypertensives-Diuretics, ACE inhibitors,Digoxin
• Anticoagulants-Aspirin, Warfarin should be provided once Atrial Fibrillation intervenes with a
target INR of 2–3.
• Surgery-mitral valve replacement

18. Aortic stenosis
• Outflow obstruction leads to an increase in left ventricular (LV) systolic pressure.
• To compensate , LV wall thickness increases by parallel replication of sarcomeres, producing
concentric hypertrophy.
• At this stage, the chamber is not dilated and ventricular function is preserved, although diastolic
compliance is reduced.
• Eventually, LV end-diastolic pressure (LVEDP) rises ,causing a corresponding increase in
pulmonary capillary arterial pressures and a decrease in cardiac output due to diastolic dysfunction.
• The contractility of the myocardium may also diminish, which leads to a decrease in cardiac output
due to systolic dysfunction.

19. Aortic Stenosis Management
• Severe AS (valve area <1 cm2), strenuous physical activity and competitive sports should be
avoided, even in the asymptomatic stage.
• Avoid dehydration and hypovolemia to protect against a significant reduction in CO.
• Antihypertensives - Diuretics and ACE inhibitors.
• Percutaneous Balloon aortic valvuloplasty.
• Aortic valve replacement.

20. Aortic Regurgitation
• Acute AR of significant severity leads to:
 increased blood volume in the LV during diastole. The LV does not have sufficient time to dilate in
response to the sudden increase in volume.
 LV end-diastolic pressure increases rapidly, causing an increase in pulmonary venous pressure.
• Chronic AR causes:
 Gradual left ventricular volume overload
 LV enlargement
eccentric hypertrophy.
The LV becomes larger and more compliant, with greater capacity to deliver a large stroke volume
that can compensate for the regurgitant volume

21. Aortic Regurgitation-Management
• Antihypertensives- diuretics and vasodilators
• Beta blockers are also best avoided so as not to reduce the CO further or slow the heart rate.
• Surgery is the treatment of choice-Aortic valve replacement.

22. Tricuspid regurgitation
• The pathophysiology of tricuspid regurgitation focuses on the structural incompetence of
the valve.
• Tricuspid valve insufficiency due to leaflet abnormalities may be secondary to
endocarditis or rheumatic heart disease.
• Ebstein anomaly is the most common congenital form of tricuspid regurgitation.
• Chronic RV volume overload results in right-sided congestive heart failure (CHF)
manifested by hepatic congestion, peripheral edema, and ascites.
• In severe TR, the CO is usually markedly reduced
• The mean RA and the RV end-diastolic pressures are often elevated.
• Systolic murmur best heard at the left lower sternal border

23. TR-management
• Antihypertensives-ACE inhibitors,Lasix,Aldactone,Digoxin
• Tricuspid valve annuloplasty
• Tricuspid replacement may rarely be required for severe, primary TR.

24. Prevention
• Primary prophylaxis (initial course of antibiotics administered to eradicate the streptococcal
infection.
• An injection of 0.6-1.2 million units of benzathine penicillin G intramuscularly every 4 weeks is
the recommended regimen for secondary prophylaxis upto 21 years of age.
• Prophylactic antibiotics 1 hour before surgical or dental procedures.