Sudden Death Caused by Aortic Dissection in a Patient with Polycystic Kidney Disease

Summary: Sudden death caused by aortic dissection in a patient with polycystic kidney disease: A 43-year-old man presented at the emergency medical unit with chest pain. The results of a clinical examination were normal, apart from sternum pain (without radiation) on palpation. The patient had no respiratory problems and the pain was relieved by paracetamol. The electrocardiogram, laboratory tests and chest X-ray were normal. However, the man was found dead the next morning. In the autopsy, we noted the presence of haemopericardium, aortic dissection (starting from the vessel's origin and extended to the aortic arch and on through the diaphragm), polycystic kidney disease and liver cysts. In adult autosomal dominant polycystic kidney disease (ADPKD) patients, the main causes of death are ruptured intracerebral aneurysms, coronary artery disease, congestive heart failure, valvular heart disease and ruptured abdominal aortic aneurysms. Aortic dissection is considered to be rare cause of sudden death in ADPKD sufferers. ADPKD can have serious consequences for the vascular system. The families of confirmed ADPKD sufferers must be informed and screened as early as possible, in order to prevent renal and cardiovascular complications. Key-words: Sudden death - Polycystic kidney disease -Aortic dissection - Chest pain. ________________________ INTRODUCTION Medicolegal autopsies are typically performed in cases of suspected homicide, medical malpractice and accidents (including road accidents), in order to clarify the causes of death and establish the identity. In French court cases, the results of a prosecutor-requested autopsy are not published by the prosecutor's office. However, when hereditary diseases are identified, the family of the deceased must be informed rapidly, in order to perform screening as soon as possible and implement treatment if required. CASE REPORT A 43-year-old man presented at our emergency medical unit with chest pain. Treated hypertension was the only element of note in his personal medical history. The results of the clinical examination were normal, apart from sternum pain (without radiation) on palpation. The patient had no respiratory problems and the pain was relieved by paracetamol. Laboratory tests (including serum creatinine and troponin levels) were normal and the results of a chest X-ray and an electrocardiogram were unremarkable. Based on a diagnosis of parietal pain, the patient was provided with symptomatic medication and discharged from hospital. The next morning, the man was found dead in his room by his son. The public prosecutor requested an autopsy to determine the cause of death. A post-mortem, whole-body CT scan was performed prior to the autopsy and revealed pericardial effusion ranging from 2 to 3,5 cm in thickness (Fig. 1). Bilateral polykystosis of the kidneys was observed (Fig. 2). Hepatomegaly was clearly visible in liver segments I and IV. The external examination was unremarkable. During the autopsy, we found haemopericardium; it consisted of 260 ml of blood and many clots (total weight: 631 grams). There was a left posterior mediastinal haematoma (4 cm ? 2 cm) starting from above the left mainstem bronchus. We noticed aortic dissection, which started from the vessel's origin and extended to the aortic arch and on through the diaphragm. The dissection affected each brachiocephalic trunk to a similar extent. The aorta's initial diameter was 23 mm. The liver weighed 2635 grams and there were cysts (5 mm in diameter) in the left lobe. On dissection, we found a 1 cm cyst in the left lobe and a 2 cm cyst in the right lobe. The right kidney weighed 1090 grams (dimensions: 21 cm ? 12 cm ? 9 cm) and contained many cysts. In histological sections, the largest cysts measured 8 cm in diameter and contained a clear liquid. The left kidney weighed 980 grams (dimensions: 23 cm ? 10 cm ? 9 cm) and was also polycystic (Fig. …