Rheumatic Heart Disease: Causes, Symptoms, Diagnosis and Treatment

Acute rheumatic fever (ARF) results due to a complex interaction between group A streptococcus (GAS) and a susceptible host, in a stage set by environmental factors. This process commonly affects the joints and heart. Cardiac involvement occurs in the form of carditis, which can be of variable severity, and results in significant morbidity. The acute rheumatic carditis may resolve completely or persist and evolve into a chronic process of rheumatic heart disease (RHD).

What causes rheumatic heart disease?

Person with heart pain

Though the global burden might have declined, in India, RHD continues to be a significant public health problem. As per the research, India has around 40% of the global RHD population. Increased prevalence in developing countries is attributed to low socio-economic status, overcrowding, poor nutrition, poor sanitation and lack of access to health care. Indian states with strong primary health care infrastructure (e.g., Kerala) have a lower RHD burden than other states

What are the symptoms of rheumatic heart disease?

child with thermometer

Joint Manifestations

It is an early manifestation, seen in 40–70% of ARF episodes. The classical description is that of “migratory polyarthritis” involving large joints (knees, ankle, elbow, wrists). However, such migratory polyarthritis is becoming uncommon, and monarthritis and arthralgias are more common especially among patients with recurrences.  Arthritis has the least specificity among all manifestations.

Carditis

Carditis is an early manifestation, with 80% of patients developing it in the first 2 wk of ARF. Tachycardia is an early sign of carditis. Mitral valve is commonly involved and mitral regurgitation (MR) is the commonest abnormality. Aortic regurgitation can also occur, but usually occurs in association with MR. Severity of carditis may be variable, ranging from an asymptomatic patient (mild MR) to a critically ill patient with dyspnea, palpitations, heart failure (ruptured chordae causing in acute severe MR).

Although classically described as “pancarditis”, endocarditis is the most dominant involvement. Pericarditis occurs in only a few of ARF patients. Clinical carditis is diagnosed in 50–70% of cases, whereas echocardiography can diagnose an additional 12–21% of cases.

Other Major Manifestations

Chorea (Sydenhams chorea) is a neuropsychiatric syndrome commonly affecting females. It is characterized by involuntary, purposeless movements and emotional lability. Chorea is a late manifestation and can occur till 6 months after a GAS pharyngitis. Rarely, chorea may be the sole manifestation of ARF.

Subcutaneous nodules are round, firm and non-tender nodular lesions, occurring in crops over bony prominences (elbows, wrists, knees, ankles, spinous process), and scalp. They are seen in less than 10% of ARF episodes. Erythema marginatum is identified as a painless, bright pink maculopapular rash, with serpiginous borders and central clearing. These are evanescent lesions occurring in trunk and proximal extremities and seen very rarely . Both these cutaneous manifestations are usually associated with carditis.

How is rheumatic heart disease diagnosed?

doctors diagnosis

Lower thresholds for joint manifestations, fever etc. are adopted for the high risk population. These modifications are made to improve the sensitivity of the criteria in moderate/high risk populations. Echocardiogram, ECG, chest- x ray, blood tests can be done. An MRI can also be conducted to detect this.

Treatment and prevention of rheumatic heart disease

pills in hand

The management of ARD focusses on 4 goals: 1) Treatment of GAS pharyngitis, 2) Treatment of ARF manifestations, 3) Prevention of recurrences of ARF, and 4) Education of the patient and family. Treatment of ARF begins with measures to eradicate GAS carriage, irrespective of symptoms of sore throat.

Penicillin is the preferred antibiotic for this purpose since most of the GAS strains are penicillin sensitive. Symptomatic supportive care is the mainstay therapy for ARF. Bed rest and limited physical activity is recommended for 4–6 wk in case of carditis. Fever responds to paracetamol and aspirin; however, when in doubt, aspirin is usually avoided since it may mask some of the features and hamper the clinical diagnosis of ARF. Anti-inflammatory therapy in the form of nonsteroidal anti-inflammatory drugs (NSAIDs) or steroids are initiated.

Prednisolone is the preferred steroid in patients with carditis, and administered at 2 mg/kg/d in patients with carditis. Duration of therapy is usually for 4 wk, but may be extended up to 12 wk in patients with severe carditis. Aspirin is the preferred NSAID and prescribed at a dose of 180–100 mg/kg/d in three to four divided doses. Naproxen was shown to have similar effect as that of aspirin.

Steroids result in faster resolution of inflammation, normalization of ESR than aspirin, however there is no robust data to support the unequivocal benefit of these anti-inflammatory therapies in preventing the progression to RHD.

Medical Therapy:  Patients who develop chronic RHD mostly remain stable for a long period of time and need to be placed on medical therapy. Patients with MR may benefit from afterload reduction with angiotensin convertase enzyme (ACE) inhibitors. Anticoagulation is recommended in MS patients with atrial fibrillation (AF), history of prior thromboembolic events or left atrial thrombus.

Patients who are symptomatic may be ameliorated with diuretic therapy to relieve pulmonary venous congestion. Heart rate control with beta blockers may benefit patients with MS, especially those with AF, by increasing the diastolic time and improving cardiac output.

However, medical therapy in these symptomatic RHD patients is only a temporizing measure and they should be referred for surgery unless contraindicated. The timing of intervention in children has been extrapolated from adult guidelines

Rheumatic heart disease is a preventable cardiovascular disease. Improvement in living conditions is the most effective method to decrease the burden of ARF and RHD. Preventing recurrences of ARF by long-term antibiotic prophylaxis help decrease the progression and severity of RHD. Creating awareness among the population regarding the importance of antibiotic prophylaxis may improve adherence rates. The techniques of mechanical intervention of established RHD also continue to evolve, with improvement in outcomes.