Rheumatic Heart Disease

Permanent heart damage due to rheumatic fever is known as chronic rheumatic heart disease
• During a first rheumatic fever attack, about half of people develop heart inflammation,
but this doesn't always result in permanent damage.
• Most people with rheumatic fever recover fully after six weeks
• Chronic Rheumatic Valvular heart disease develops in at least half of those affected by rheumatic fever with carditis. Two-thirds of cases occur in females.
• History of rheumatic fever is only elicited in about half of all patients with chronic rheumatic heart disease.
• In some cases, however, one or more of the heart valves may be scarred.
• The mitral valve is affected in more than 90% of cases. The aortic valve is the most frequently affected, followed by the tricuspid and then pulmonary valve.
• Isolated mitral stenosis accounts for about 25% of all cases of rheumatic heart disease.
An additional 40% of all cases of rheumatic heart disease have mixed mitral stenosis and
regurgitation.
• Valve disease may be symptomatic during fulminant forms of acute rheumatic fever but
may remain asymptomatic for many years.
• In rare cases of rheumatic fever, the inflammation causes so much damage to the heart
the muscle that it leads to heart failure.
• If there is a serious impairment of the function of affected heart valves, surgery may be needed to repair or replace the damaged valve or valves.
Pathology
• In contrast to the destructive process of acute rheumatic fever (ARF), the main pathological process in chronic rheumatic heart disease is progressive fibrosis.
• The heart valves are predominantly affected but the involvement of the pericardium and the myocardium may contribute to heart failure and conduction disorders.
• Fusion of the mitral valve commissures and shortening of the chordae tendinea may lead
to mitral stenosis with or without regurgitation.
• Similar changes in the aortic and tricuspid valves produce distortion and rigidity of the
cusps, leading to stenosis and/or regurgitation.
• Once a valve has been damaged, the altered hemodynamic stresses perpetuate and extend the damage, even in the absence of a continuing rheumatic process.
Clinical Features and Investigations of Chronic Rheumatic Heart Disease
 
Clinical Features of Chronic Rheumatic Heart Diseases
• Breathlessness due to pulmonary congestion
• Fatigue caused by low cardiac output
• Oedema, ascites due to right heart failure
• Palpitation
• Haemoptysis that may be caused by pulmonary congestion or pulmonary embolism
• Cough due to pulmonary congestion
• Chest pain that might be contributed by pulmonary hypertension
• Symptoms of thromboembolic complications e.g. stroke, ischemic limb or deep vein thrombosis (DVT)
• Features of underlying valvular lesions (mitral regurgitation/stenosis and aortic regurgitation/stenosis
Investigation
• Chest radiography to rule out cardiomegaly
• Electrocardiogram: a prolonged PR interval
• Echocardiogram to assess the structure of the heart
• Doppler detects and quantifies regurgitation
• Cardiac catheterization (done before surgery)
Treatment of Chronic Rheumatic Heart Disease
• At the primary health care facility level, pre-referral management should be given before referring the patient for definitive diagnosis and treatment of the complications.
• These may include the provision of diuretics (when available) for those with heart failure and an initial antibiotic dose (when available).
• At the hospital level, medical therapy in rheumatic heart disease is directed toward
o Eliminating the group A streptococcal pharyngitis (if still present)
o Suppressing inflammation from the autoimmune response
o Providing supportive treatment for congestive heart failure
o Preventing and treating infective endocarditis
• Patients with chronic rheumatic heart disease require antibiotic prophylaxis before certain surgical and dental procedures to prevent bacterially endocarditis
• Oral Penicillin-V remains the drug of choice for treatment of group ‘A’ streptococcal
pharyngitis. Other drugs that may be used include Erythromycin, first-generation
Cephalosporins, Amoxycillin-clavulanate and Clarithromycin.
• When heart failure persists or worsens after aggressive medical therapy for acute rheumatic heart disease, surgery to decrease valve insufficiency may be life-saving. This intervention is done in more specialized hospitals.
• Diet to patients with rheumatic heart disease should be nutritious and without restrictions except in the patient with congestive heart failure, whose fluid and sodium intake should be restricted.
• Potassium supplementation may be necessary because of the mineralocorticoid effect of corticosteroid and the diuretics (if used).
• Patients with mild valvular lesions and minor symptoms should be treated medically. Patients with more symptoms (moderate to severe valvular) lesions are treated surgically by valvuloplasty or valve replacement. 

                                                                     
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