LAUSR

Myocardial disorders in the neonate could be a significant cause of morbidity and mortality. The neonatal myocardium is immature and undergoes several changes after birth. These include changes in the size of the myocardium, cellular transport of calcium, and utilization for fatty acid and glucose metabolism. Neonatal myocardium relies heavily on the heart rate to improve cardiac output. Myocardial disorders in the neonate can be classified as primary and secondary. Primary myocardial disorders have an inherent abnormality in the cardiac muscle and can be further subclassified based on the morphology and presentation. These include hypertrophic cardiomyopathy, dilated cardiomyopathy, and restrictive cardiomyopathy. Secondary myocardial disorders are usually caused by a systemic disorder that affects the cardiac muscle and function. These include inborn errors of metabolism, neuromuscular disorders, and mitochondrial disorders. The diagnosis and management of cardiomyopathy is very specific to the type of cardiomyopathy or underlying disorders. A team approach, including neonatology, genetics and metabolism, and cardiology and cardiac transplantation, is essential in managing these cases.