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Cardiovascular disorders are the most common cause of mortality in autosomal dominant polycystic kidney disease (ADPKD). This review considers recent clinical and basic science studies that address the contributing factors of cardiovascular dysfunction in ADPKD. In particular, attention is placed on how dysfunction of the polycystin proteins located in the cardiovascular system contributes to extrarenal manifestations of ADPKD. Keywords: cardiovascular dysfunction, calcium signaling, heart failure, polycystic kidney disease