Treatable cardiomyopathies

Abstract

Cardiomyopathy is defined as primary myocardial dysfunction which is not due to hypertensive, valvular, congenital, coronary or pulmonary vascular disease. This term usually denotes a dismal prognosis short of cardiac transplantation. However, several organic diseases of the heart can result in right or left ventricular dysfunction resulting in congestive heart failure and prompting the physician to label them as cardiomyopathy; the etiological factor is overlooked as it produces very subtle features. Therefore, before labelling any child as cardiomyopathic, all possible causes of ventricular dysfunction must be excluded by clinical and investigative means. The causes of “treatable cardiomyopathy” include mechanical factors as critical aortic stenosis and pulmonic stenosis, severe coarctation of aorta in an infant and aortaarteritis is an older child. Some of the persistent arrhythmias like atrial tachycardia, fibrillation, paroxysmal junctional re-entrant tachycardia are also known for causing ventricular dysfunction producing tachycardiomyopathy. Treatment of arrhythmia improves the ventricular function. Myocardial ischemia as a result of congenital coronary anomaly (commonest being anomalous origin of left coronary artery from pulmonary artery) can also present with a cardiomyopathy like picture. Early surgical correction is very rewarding. Finally, some of the metabolic conditions like creatinine and thiamine deficiency can also produce ventricular dilatation and dysfunction. In conclusion, the so called cardiomyopathy like picture can be produced because of several reasons and an attempt must be made to identify them.