David Operation in a patient with LDS and beta-thalassemia minor: A Case Report

Abstract

Abstract We report about a 36-year-old European female patient who presented a growing ectasia of the ascending aorta of 40 mm, a sinus of valsalva of 47mm and a moderate aortic insufficiency. Also, she had an additional aneurysm in the truncus brachiocephalicus and multiple aneurysma in the arteria mammaria dextra. She was diagnosed as a Loeys-Dietz syndrome Typ RII because of a TGF-BETA receptor mutation. Additional she suffered from a beta-Thalassemia minor with hemoglobin levels of around 9 g/dL. During cardiac surgery the patient had to be cooled to at least 30°C to perform open aortic arch operation safely. This case report describes the effects of cooling, rewarming and the use of the cardiopulmonary bypass (CPB) during an extensive operation in a patient with beta-thalassemia minor.