左上大静脈遺残を伴うStanford A型急性大動脈解離を発症したTurner症候群の1例

Stanford Type A Acute Aortic Dissection with Persistent Left Superior Vena Cava in a Turner Syndrome Patient：Report of a Case

はじめに　Turner症候群はX染色体異常に起因する先天性異常であり，性腺発育不全，低身長をはじめとする身体奇形のほかに，さまざまな心血管病変を合併することが報告されている．われわれは，Turner症候群患者において左上大静脈遺残（PLSVC）を伴うStanford A型急性大動脈解離を経験したので，文献的考察を加えて報告する．

A 48-year-old woman who was diagnosed with Turner syndrome in her childhood presented with sudden onset of low back pain and respiratory discomfort. Contrast enhanced computed tomography scan revealed Stanford type A acute aortic dissection with persistent left superior vena cava （PLSVC）. Emergency ascending aortic replacement was performed. After cardiopulmonary bypass was established through cannulating right femoral artery and right superior vena cava, inferior vena cava, another venous cannula was directly placed into the left superior vena cava. After core cooling, the right atrium was incised for retrograde cardioplegia. At a tympanic temperature of 25 ℃, circulatory arrest was started and retrograde cerebral perfusion was performed through right and left superior vena cava. Her postoperative course was uneventful.