Hypertrophic cardiomyopathy is a rare disorder in infancy. Signs of myocardial ischemia and cardiomegaly are the predominate manifestations of this lesion. The spectrum of the etiology and management of hypertrophic cardiomyopathy in infancy have been updated in the past several decades. Long-term small-dose digoxin combined with prednisone, supplemented by the angiotensin-converting enzyme inhibitor captopril is an accepted therapy for endocardial fibroelastosis in infancy. Treatment with recombinant human α-glucosidase enzyme replacement therapy can reverse the electrocardiographic changes of infantile Pompe’s disease. Hypertrophic cardiomyopathy in infants of diabetic mothers is usually benign and transient, and treatment is not needed unless heart failure occurs. Differential diagnosis of hypertrophic cardiomyopathy from congenital heart defects is important for subsequent management.
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