An Unusual Cardiac Manifestation in Autosomal Dominant Polycystic Kidney Disease

Autosomal dominant polycystic kidney diseaseis a common hereditary disorder characterized by renal and extrarenal, cystic and noncystic manifestations. Connective tissue defects, including cerebral aneurysm, meningeal diverticula, abdominal wallhernias, intestinal diverticula, and cardiac valvular abnormalities, are widely known manifestations. Instead intracardiac aneurysms have never been reported in adults with autosomal dominant polycystic kidney disease. We describe a 65-year-old patient with end-stage renal disease due to autosomal dominant polycystic kidney diseaseand an atrial septumaneurysm associated with platypnoea-orthodeoxia syndrome.