ABSTRACT
ABSTRACT Objective: To report comprehensively the clinical features and the management strategies of hypertrophic cardiomyopathy in infancy. Methods: Comprehensively retrieved studies published from 2000 to present constituted the study materials for this article. Results: Signs of myocardial ischaemia and cardiomegaly are the predominate manifestations of this lesion. The spectrum of the aetiology 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 capto-pril, is an accepted therapy for endocardial fibroelastosis in infancy. The treatment with recombinant human α-glucosidase enzyme replacement therapy can reverse the electrocardiographic changes of infantile Pompe 's disease. Conclusion: Hypertrophic cardiomyopathy in infants of diabetic mothers is usually benign and transient, and the treatment is not needed unless heart failure occurs. Differential diagnosis of hypertrophic cardiomyopathy from congenital heart defects is important for subsequent management.