One of the main reasons for mortality in people with autosomal dominant polycystic kidney disease (ADPKD) is heart disease. In patients with ADPKD, left ventricular hypertrophy (LVH) is a significant and early consequence.

Twenty years ago, it was shown that as many as 46% of hypertensive ADPKD patients had LVH on echocardiography. Recent research employing cardiac magnetic resonance imaging suggested that there may be a decreased incidence of LVH in ADPKD patients. It is debatable how often LVH is in ADPKD patients. There was proof that LVH in ADPKD patients is caused by reasons other than hypertension. Studies have revealed that young adults and children with ADPKD who have well-controlled blood pressure have greater left ventricular mass indices than controls, and LVH is more common in these patients.

The intracellular signaling pathways controlled by polycystins PC-1 and PC-2 may have an impact on heart function. Independent of renal function or blood pressure, disturbances of PC-1 or PC-2 in the heart can result in significant alterations in cardiac anatomy and function. Both PC-1 and PC-2 can affect the mitophagy and autophagy processes involved in LVH, as well as the mammalian target of rapamycin.

Angiotensin-converting enzyme gene polymorphisms could contribute to LVH in ADPKD. Beyond hypertension, the pathogenesis of LVH in ADPKD will be covered in detail in this review.