Severe Aortic Stenosis and Partial Anomalous Pulmonary Venous Connection in a Turner Syndrome Patient

Abstract

A 42-year-old woman with Turner syndrome was admitted to our hospital due to severe aortic stenosis. Transthoracic echocardiography demonstrated severe aortic stenosis with a bicuspid aortic valve. Enhanced computed tomography revealed that the left upper pulmonary vein connected to the innominate vein, and the ascending aorta was enlarged (maximum diameter of 41 mm). Surgical intervention was performed though median sternotomy with cardiopulmonary bypass. After achieving cardiac arrest by antegrade cardioplegia, we performed an anastomosis to connect the left upper pulmonary vein to the left atrial appendage. Then, aortic valve replacement was performed with an oblique aortotomy in the anterior segment of the ascending aorta. The aortic valve was a unicaspid aortic valve. Following completion of aortic valve replacement with a mechanical valve, reduction aortoplasty was performed on the ascending aorta. The postoperative course was uneventful.