Ebrahimi Niloufar, Caliskan Yasar, Garimella Pranav S, Carriazo Sol, Chebib Fouad T, Bateni Giv Heidari, Dahl Neera K, Rastogi Anjay, Abdipour Amir, Norouzi Sayna
Department of Medicine, Division of Nephrology, Loma Linda University Medical Center, Loma Linda, California, USA.
Saint Louis University Center for Abdominal Transplantation, Saint Louis University, St. Louis, Missouri, USA.
Kidney Int Rep. 2025 Jul 5;10(9):2953-2966. doi: 10.1016/j.ekir.2025.06.054. eCollection 2025 Sep.
Autosomal dominant polycystic kidney disease (ADPKD), primarily caused by mutations in the or gene, is among the most common hereditary kidney diseases worldwide and is associated with significant extrarenal manifestations, including cardiovascular disease. In patients with ADPKD, cardiovascular disease is the major cause of mortality and is associated with a high burden of comorbidities. Cardiovascular manifestations include hypertension, which may lead to left ventricular hypertrophy (LVH) and diastolic dysfunction, as well as valvular abnormalities, aortic aneurysm, and pericardial effusion (PCE). The cardiovascular manifestations can present early as part of the ADPKD manifestation or later because of chronic kidney disease (CKD) progression. Early detection of cardiovascular manifestations can play a pivotal role in better management of these patients. Hypertension in ADPKD might start at an early age and is driven by a complex interplay of polycystin (PC) dysfunction, intracellular signaling disruptions, and activation of the renin-angiotensin-aldosterone system (RAAS), which can contribute to vascular structural changes and impaired endothelial function. Valvular involvement exhibits a bimodal pattern of age distribution, with early manifestations occurring in younger patients likely linked to genetic factors and later complications emerging as part of CKD progression. This review explores cardiovascular complications in ADPKD, emphasizing the need for early detection, and briefly provides an overview of tailored management approaches to improve outcomes in this high-risk population.