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Acromegaly is a rare endocrine disorder that carries a significant burden of cardiovascular morbidity and mortality. Abnormalities of the growth hormone/insulin-like growth factor-1 axis in acromegaly lead to the characteristic cardiovascular manifestations of this disease. One hallmark feature of the disease is acromegalic cardiomyopathy, a syndrome of progressive cardiac dysfunction characterized by left ventricular hypertrophy, diastolic dysfunction, and combined systolic and diastolic dysfunction in the very advanced stage. In this article, we review the current concepts regarding the pathophysiology of acromegalic cardiomyopathy and discuss the various risk factors for its development. We also explore the question of whether the alterations in cardiac function are reversible with successful biochemical control of growth hormone excess either medically or surgically.