Dissection of the Thoracic Aorta in a Patient with Autosomal Dominant Polycystic Kidney Disease

Abstract

Internal Medicine,Gulhane Military Medical Academy, Haydarpasa Training Hospital, Istanbul, Turkey.Autosomal dominant polycystic kidney disease(ADPKD) is a systemic illness with a number of extra-renal manifestations. A 61-year-old male patient withknown ADPKD was admitted to the emergency roomof our hospital in May 2003 with severe chest painradiating to his neck and back, and a sense ofbreathlessness. There was no history of trauma.On admission, he was hypotensive (90/50 mmHg)and his heart rate was 110 beats/min. He had a mid-systolic murmur, which was most marked at the leftsternal edge. His peripheral pulses were weak. Bilater-al basal crepitations were found on pulmonaryexamination. Abdominal examination was normalexcept for large palpable polycystic kidneys. He hadno focal neurologic signs. Laboratory examinationshowed no significant abnormalities except for raisedserum urea (121 mg/dL) and creatinine (4.1 mg/dL).An electrocardiogram showed depression of ST seg-ments in the anterior leads. Emergency computerizedtomography scanning of the thorax and abdomen de-monstrated dissection and intramural thrombus withinthe descending thoracic aorta and bilateral polycystickidneys (Panels A and B).He was transferred immediately for aortic surgery.During the operation, a type II thoracic aortic dissectionwas observed. His recovery was complicated by theexpansion of the dissection and he died on the thirdpostoperative day.Numerous cardiovascular abnormalities have beenreported in ADPKD patients, including heart valvelesion, cerebral aneurysm, splenic artery aneurysm,aortic root dilatation, abdominal aortic aneurysm,thoracic aortic dissection, and cervical artery dissection[1–3]. The prevalence of intracranial aneurysm andrupture are five-fold higher in ADPKD patients than inthe general population [4]. However, the prevalence ofextracerebral aneurysms is not clear. One autopsy seriesdescribed aortic dissection as being seven times morecommon in patients with ADPKD than in the generalpopulation [2]. Spontaneous artery dissection wasreported as a major complication in five unrelatedADPKD patients. All underwent spontaneous dissect-ion and none had phenotype of elastic tissue disorderor thoracic aortic dissection. Torra et al, in their case-control study, found a similar distribution of aorticdiameters in 139 ADPKD patients and their 149unaffected relatives [5]. The case reported by Paynteret al appears to be very similar to our case [3]. Arterialdissection may belong to the spectrum of extrarenalmanifestations of ADPKD. The association of ADPKD