A CASE OF TURNERS-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 wa s 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 t o our hospital in June 1994 as echocardiography revealed rapid dilatat ion of ascending aorta and aortic regurgitation. A chest X-ray showed cardiothoracic ratio of 63% and transesophageal echocardiogram reveale d that ascending aortic diameter was extended up to 60 mm at its maxim um and that it was possible to distinguish true lumen from false lumen . The aortic arch was found to be normal. Also revealed by cardiac cat heterization was drainage of the left upper pulmonary vein to the inno minate 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 ost iums of the coronary arteries were directly anastomosed to the composi te graft with Carrel patch. After declamp of the aorta, the left pulmo nary vein was directly anastomosed to the left atrial appendage withou t causing stenosis. The postoperative course was uneventful, and the c ineangiogram after surgery demonstrated successful repair. Reports of cases of Turner's syndrome like this are sparse.