先天性心膜欠損により心タンポナーデを免れた急性大動脈解離の1例

Acute Aortic Dissection Escaping Cardiac Tamponade due to Congenital Pericardial Defect：Report of a Case

はじめに　先天性心膜欠損症は発症率0.01％程度と非常にまれであり，左側心膜欠損が多いとされる．一方，Stanford A型急性大動脈解離の致死的な合併症として，心タンポナーデによる循環不全がある．われわれは，Stanford A型急性大動脈解離を発症したものの左側心膜欠損のために心タンポナーデからの循環不全の進展を免れ，手術により救命できた症例を経験したので報告する．

The natural course of Stanford type A acute aortic dissection （AAAD） has a poor prognosis. Early diagnosis is crucial, but in clinical practice some patients do not have typical symptoms, leading to a delay in diagnosis. We encountered a patient who complained only of shoulder pain and moderate respiratory distress. A chest computed tomography （CT） examination showed a dilated ascending aorta and a massive left hemothorax with minimal pericardial effusion. Intraoperative findings revealed aortic dissection of the ascending aorta and a congenital defect on the left pericardium. We performed graft replacement of the aortic root and ascending aorta. Usually, cardiac tamponade is a fatal complication of AAAD. However, in this case, the congenital pericardial defect drained the hemorrhage into the thoracic cavity and relieved cardiac tamponade. AAAD with a congenital pericardial defect may present clinically atypical. In this case, the patient could be saved by surgery without developing circulatory failure due to cardiac tamponade.