A case of Turner's syndrome associated with partial anomalous pulmonary venous return complicated by dissecting aortic aneurysm and aortic regurgitation

We report a successful surgical case with Turner's syndrome associated with partial anomalous pulmonary venous return (PAPVR) complicated by aortic dissection and aortic regurgitation without coarctation of the aorta. The patient, a 30-year-old woman, is of a short stature who was diagnosed with Turner's syndrome at the age of 12. She has suffered from dyspnea and edema of the legs since a year ago and was admitted to our hospital in June 1994 as echocardiography revealed rapid dilatation of ascending aorta and aortic regurgitation. A chest X-ray showed cardiothoracic ratio of 63% and transesophageal echocardiogram revealed that ascending aortic diameter was extended up to 60 mm at its maximum and that it was possible to distinguish true lumen from false lumen. The aortic arch was found to be normal. Also revealed by cardiac catheterization was drainage of the left upper pulmonary vein to the innominate vein. The L-R shunt ratio was 2.2. The surgery was performed by the Bentall method. The composite graft with a 21 mm St. Jude Medical prosthetic heart valve placed on the annulus of aortic valve. The ostiums of the coronary arteries were directly anastomosed to the composite graft with Carrel patch. After declamp of the aorta, the left pulmonary vein was directly anastomosed to the left atrial appendage without causing stenosis. The postoperative course was uneventful, and the cineangiogram after surgery demonstrated successful repair. Reports of cases of Turner's syndrome like this are sparse.