A doença renal policística autossômica dominante (DRPAD), uma das anormalidades genéticas mais comuns, é responsável por 10% dos casos de insuficiência renal crônica terminal. O acometimento extrarrenal da DRPAD manifesta-se por cistos no fígado e pâncreas, aneurismas intra e extracranianos, e anormalidades cardíacas valvares, comprometendo a raiz da aorta e as valvas aórtica e mitral. Neste artigo descrevemos o caso de um paciente com aneurismas das artérias coronárias no contexto de outras manifestações sugestivas de DRPAD.

Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common genetic abnormalities and is responsible for over 10% of end-stage chronic kidney disease cases. Extrarenal involvement of ADPKD is manifested by liver and pancreatic cysts, intra and extracranial aneurysms and valvular heart disease, involving the aortic root and the aortic and mitral valves. In this article we report a case of a patient with large coronary artery aneurysms in the context of other manifestations suggesting ADPKD.