Health Awareness blog

Rheumatic heart disease is the serious complication of rheumatic fever. Acute rheumatic fever follows 0.3% of cases of group A beta haemolytic streptococcal pharyngitis in children. Up to 39% of all patients with rheumatic fever may develop various degrees of pancarditis. This is associated with valve insufficiency, heart failure and even death.
With chronic rheumatic heart disease, patients develop valve stenosis with varying degrees of regurgitation, atrial dilation, arrhythmias, and ventricular dysfunction. Chronic rheumatic heart disease remains the leading cause of mitral valve stenosis and valve replacement in adults in the United States.

INCIDENCE
Age for the rheumatic heart disease is same with rheumatic fever, between 5 to 15 years old. Median age is 10 years old. However, about 20% of cases occur during adulthood. Prevalence of disease in male and female is equal, but the prognosis is poor in female rather than male. Although the incidence of rheumatic heart disease is reduced in developed country, it still remains a major health problem worldwide. Estimated occur in 5 – 30 million children and young adult. The mortality rate is about 1 in 10. There is not documentation that race has any influence to the disease incidence.

PATHOPHYSIOLOGY
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, which then 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.

GROUP A BETA HAEMOLYTIC STREPTOCOCCAL (GABHS)

Group A Streptococcus is a gram-positive coccus that frequently colonizes the skin and oropharynx. This organism may cause suppurative disease, such as pharyngitis, impetigo, cellulitis, myositis, pneumonia, and puerperal sepsis. It also may be associated with non suppurative disease, such as rheumatic fever and acute post streptococcal glomerulonephritis. Group A streptococci elaborate the cytolytic toxins streptolysins S and O. Of these, streptolysin O induces persistently high antibody titers that provide a useful marker of group A streptococcal infection and its non suppurative complications.
Group A Streptococcus, has a group A carbohydrate antigen in the cell wall that is composed of a branched polymer of L- rhamnose and N- acetyl-D-glucosamine in a 2:1 ratio. Strains that produces rheumatic fever are M types l, 3, 5, 6, 18 & 24.
Pharyngitis that is caused by GABHS can lead to acute rheumatic fever , rheumatic heart disease & post streptococcus glomerulonepritis while skin infection that is caused by GABHS leads to post streptococcal glomerulonephritis only. It will not result in rheumatic fever or carditis as skin lipid cholesterol inhibits antigenicity.

DIAGNOSIS
A diagnosis of rheumatic heart disease is made after confirming antecedent rheumatic fever. The modified Jones criteria (revised in 1992) provide guidelines for the diagnosis of rheumatic fever. After a diagnosis of rheumatic fever is made, symptoms consistent with heart failure, such as difficulty breathing, exercise intolerance, and a rapid heart rate out of proportion to fever, may be indications of carditis and rheumatic heart disease.

CARDIAC MANIFESTATIONS OF ACUTE RHEUMATIC FEVER
Cardiac manifestations of acute rheumatic fever are pancarditis, new or changing murmur, congestive heart failure or pericarditis.

Pancarditis
• It is characterized by endocarditis, myocarditis and pericarditis.
• The patients might come with symptoms of dyspnea, mild to moderate chest discomfort, pleuritic chest pain, edema, cough, or orthopnea
• Other symptoms are new murmur and tachycardia that is out of proportion to fever.
• On echocardiography, we can see mitral regurgitation particularly in association with aortic regurgitation, reduced ventricular contraction, pericardial effusion.

New or changing murmur
• Apical pansystolic murmur is a high-pitched, blowing-quality murmur of mitral regurgitation that radiates to the left axilla. The murmur is unaffected by respiration or position. Intensity varies but is grade 2/6 or greater. The mitral regurgitation is related to dysfunction of the valve, chordae, and papillary muscles.
• Apical diastolic murmur (also known as a Carey-Coombs murmur) is heard with active carditis and accompanies severe mitral insufficiency. It is related to relative mitral stenosis, as the large volume of regurgitant flow recrosses the mitral valve during ventricular filling. It is heard best with the bell of the stethoscope, while the patient is in the left lateral position and the breath held in expiration. This murmur is low pitched, rumbling, and resembles the roll of a distant drum.
• Basal diastolic murmur is an early diastolic murmur of aortic regurgitation and is high-pitched, blowing, decrescendo, and heard best along the right upper sternal border after deep expiration while the patient is leaning forward.

Congestive heart failure
• Heart failure may develop secondary to severe valve insufficiency or myocarditis.
• The physical findings associated with heart failure include tachypnea, orthopnea, jugular venous distention, rales, hepatomegaly, a gallop rhythm, and peripheral swelling and edema.

Pericarditis
• A pericardial friction rub indicates that pericarditis is present.
• Increased cardiac dullness to percussion and muffled heart sounds are consistent with pericardial effusion.
• A paradoxical pulse (drop in systolic blood pressure with inspiration) with decreased systemic pressure and perfusion and evidence of diastolic indentation of the right ventricle on echocardiogram reflect impending pericardial tamponade. In this clinical emergency, pericardial effusion should be treated by pericardiocentesis.

NON CARDIAC MANIFESTATION OF ACUTE RHEUMATIC FEVER
Non cardiac manifestation include polyarthritis, chorea, erythema marginatum, and subcutaneous nodules.

Arthritis:
• Flitting & fleeting migratory polyarthritis, involving major joints
• Commonly involved joints-knee,ankle,elbow & wrist
• Occur in 80%,involved joints are exquisitely tender
• In children below 5 yrs arthritis usually mild but carditis more prominent
• Arthritis do not progress to chronic disease

Sydenham Chorea:
• Occur in 10-15% of cases
• Mainly in girls of 1-15 yrs age
• May appear even 6/12 after the attack of rheumatic fever
• Clinically manifest as-clumsiness, incoordination, deterioration of handwriting, emotional lability or grimacing of face, uncontollable movement(exarcebated by stress and disappeared by sleep , are characteristic).
• Clinical signs- pronator sign, jack in the box sign , milking sign of hands

Erythema Marginatum:
• Occur in <3%.
• Unique,transient,serpiginous-looking lesions of 1-2 inches in size
• Pale center with red irregular margin
• More on trunks & limbs & non-itchy
• Worsens with application of heat
• Often associated with chronic carditis

Subcutaneous nodules:
• Occur in <1%
• Painless,pea-sized,palpable nodules
• Mainly over extensor surfaces of joints,spine,scapulae & scalp
• Associated with strong seropositivity
• Always associated with severe carditis

Other features:
• Fever (>38 degree celsious)
• Arthralgia
• Pallor
• Anorexia
• Abdominal pain

CARDIAC MANIFESTATION OF CHRONIC RHEUMATIC HEART DISEASE(CRHD)

Valve deformities, thromboembolism, cardiac hemolytic anemia, and atrial arrhythmias are the most common cardiac manifestation. Mitral stenosis occur in 25% of cases. Aortic stenosis also may occur. Moderate to severe carditis have persistent mitral and/or aortic regurgitation.
During acute rheumatic fever, the left ventricle is frequently dilated in association with a normal or increased fractional shortening. In chronic rheumatic heart disease, echocardiography may be used to track the progression of valve stenosis and may help determine the time for surgical intervention. The leaflets of affected valves become diffusely thickened, with fusion of the commissures and chordae tendineae. Increased echo density of the mitral valve may signify calcification.

INVESTIGATIONS AND EVALUATION:
LABORATORY STUDIES:
• Throat swab
• Rapid antigen detection
• Anti streptococcal antibodies
• C-reactive protein
• ESR
• Heart reactive antibodies – tropomyosin

IMAGING STUDIES:
• CXR-cardiomegaly,pulmonary congestion,finding associated with heart failure
• ECHO – With mild carditis, Doppler evidence of mitral regurgitation may be present during the acute phase of disease but resolves in weeks to months.

ELECTROCARDIOGRAM (ECG):
• Sinus tachycardia most frequently accompanies acute rheumatic heart disease. Alternatively, some children may develop sinus bradycardia from the increased vagal tone.
• First-degree atrioventricular (AV) block (prolongation of the PR interval) is observed in some patients.
• Acute rheumatic fever is associated with pericarditis, ST segment elevation may be present and is marked most in lead II, III, aVF, and V4 -V6.
• There may be evidence of atrial flutter, multifocal atrial tachycardia, or atrial fibrillation from chronic mitral valve disease and atrial dilation.

HISTOLOGY FINDING:
• Aschoff bodies (perivascular foci of eosinophilic collagen surrounded by lymphocytes, plasma cells, and macrophages) are found in the pericardium, perivascular regions of the myocardium, and endocardium. The Aschoff bodies assume a granulomatous appearance with a central fibrinoid focus and eventually are replaced by nodules of scar tissue.
• Anitschkow cells are plump macrophages within Aschoff bodies.
• In the pericardium, fibrinous and serofibrinous exudates may produce an appearance of “bread and butter” pericarditis.

TREATMENT OF CHRONICRHEUMATIC HEART DISEASE
Step I – primary prevention (eradication of streptococci)-If still present
Step II – anti inflammatory treatment (aspirin,steroids)
Step III- supportive management & management of complications-supportive treatment for CCF.
Step IV- secondary prevention (prevention of recurrent attacks)

STEP I: Primary Prevention of Rheumatic Fever (Treatment of Streptococcal Tonsillopharyngitis)

- Intramuscular benzathine penicillin G 600 000 U for patients 27 kg (60 lb) and
1 200 000 U for patients >27 kg
- Oral penicillin V (phenoxymethyl penicillin) 250 mg 6Hly (for children) and 500 mg 6Hly (for adolescents and adults) to take orally for 10 days.

For individuals allergic to penicillin:
Agent 1 : Erythromycin (Estolate) 20-40 mg/kg/d 2-4 times daily (maximum 1 g/d)
to take orally for 10 days
Agent 2: Ethylsuccinate 40 mg/kg/d 2-4 times daily (maximum 1 g/d) to take orally for 10 days

Step II: Anti inflammatory treatment
• Mild/no carditis: oral aspirin 80-100mg/kg/day in 4 divided dose for 2-4 weeks, taper over 4 weeks.
• Pericarditis/moderate to severe carditis: oral prednisolone 2mg/kg/day in 2 divided doses for 2-4 weeks, taper with additional of aspirin as above. (while tapering add aspirin 80mg/kg/day for 2 weeks and continue aspirin alone 100/kg/day for another 4 weeks)

Step III: Supportive management & management of complications
• Bed rest
• Treatment of congestive cardiac failure: -digitalis,diuretics
• Treatment of chorea:-diazepam or haloperidol
• Rest to joints & supportive splinting

STEP IV : Secondary Prevention of Rheumatic Fever (Prevention of Recurrent Attacks)

- Intramuscular Benzathine penicillin G 1 200 000 U every 4 weeks*
- Oral Penicillin V 250 mg twice daily
- Oral ulfadiazine 0.5 g once daily for patients 27 kg (60 lb) and 1.0 g once daily for patients >27 kg (60 lb)

For individuals allergic to penicillin and sulfadiazine:
Erythromycin 250 mg twice daily Oral

*In high-risk situations, administration every 3 weeks is justified and recommended
Duration of Secondary Rheumatic Fever Prophylaxis (American Heart association)
For rheumatic fever with carditis and residual heart disease to take prophylaxis for at least 10 years since last episode and at least until (persistent valvar disease*) age 40 years old.  Sometimes lifelong prophylaxis is necessary.

For heumatic fever with carditis but no residual heart disease, to take prophylaxis for 10 years or well into adulthood whichever is longer (no valvar disease*)

For rheumatic fever without carditis, to take prophylaxis for 5 years or until age 21 year old, whichever longer

PROGNOSIS
• Rheumatic fever can recur whenever the individual experience new GABH streptococcal infection, if not on prophylactic medicines
• Good prognosis for older age group & if no carditis during the initial attack
• Bad prognosis for younger children & those with carditis with valvar lesions

Cochrane Reviews
• Penicillin for secondary prevention of rheumatic fever – Intramuscular penicillin seemed to be more effective than oral penicillin in preventing rheumatic fever recurrence and streptococcal throat infections. Two-weekly or 3-weekly injections appeared to be more effective than 4-weekly injections.
• anti inflamatory treatment for carditis in acute rheumatic fever – There risk no benefit in using corticosteroid or intravenous immunoglobin to reduce the risk of heart valve lesion in patient with acute rheumatic fever. New randomised controlled trial in patient with acute rheumatic fever to assess the effect of corticosteroid such as oral prednisolone and intravenous methylprednisolone and other new anti inflammatory agents are warranted.
• Short versus standard duration antibiotic therapy for acute streptococcal pharyngitis in children – Three to six days of oral antibiotics had comparable efficacy compared to the standard duration 10 day oral penicillin in treating children with acute GABHS pharyngitis. In countries with low rates of rheumatic fever, it appears safe and efficacious to treat children with acute GABHS pharyngitis with short duration antibiotics. In areas where the prevalence of rheumatic heart disease is still high, our results must be interpreted with caution

Other relevant topic:

• Mnemonic for rheumatic fever
• Rheumatic fever
• Revised Jones Criteria

 

Reference:
1) Paediatric protocols for Malaysian Hospital
2) Cochrane database
3) Kliegman,Behrman:Nelson textbook of Pediatrics
4) American Heart Association

Tags: arthritis, chorea, pancarditis, pericarditis, rheumatic heart disease, subcutaneous nodules

Technorati Tags: arthritis, chorea, pancarditis, pericarditis, rheumatic heart disease, subcutaneous nodules

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