This is a rare disease in pediatric age groups.

Causes of DCMP

Anomalous origin of the left coronary artery (ALCAPA), myocarditis common causes include viral infection with coxachie, echo, HIV, measles, mumps and rubella etc.

Sometimes, cause is familial with autosomal dominant, recessive and x-linked inheritance describes.

Systemic causes: can be metabolic, endocrine, storage, mitochondrial and connective tissue disorders

Clinical Presentation

If DCMP is severe then child is generally in symptomatic heart failure. Child presents with difficulty in breathing, cough, cold, poor feeding, edema over feet etc.

Investigations

Chest x-ray shows cardiomegaly, increased pulmonary vascularity due to fluid overload in lungs alveoli, in association with liver enlargement hepatomegaly, raises doubt of DCMP.

Transthoracic echocardiography confirms the diagnosis by showing dilatation of cardiac chambers, mitral regurgitation, decreased cardiac pumping function.

In pediatric age groups, coronary arteries should be assessed very carefully as abnormalities in coronary arteries also leads to similar picture.

ECG differentiates coronary abnormalities cause from non-coronary causes of DCMP. Generally ECG shows sinus tachycardia, left atrial enlargement etc.

Treatment

Medical treatment

This gives symptomatic relief. Drugs that commonly used are diuretic, ACE inhibitors, B-blockers. Digoxin should be used very carefully in case of myocarditis.

DCMP patients are very prone for arrhythmias so anti-arrhythmic drugs are also used whenever required.

Poor LV function is always at risk for blood clot formation in cardiac chambers so anti-clot forming drugs like aspirin is also used wherever indicated.

Mechanical and surgical treatment

Left ventricular assist devices (LVAD) and biventricular assist devices, although not widely available and quite expensive will have a role at least in short term, and act as a bridge to heart transplant.

Cardiac Transplant: Indicated in end stage heart failure.

Prognosis

Death due to DCMP is highest in first year after diagnosis due to intractable heart failure. Late deaths are due to arrhythmia.

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