Concomitant Repair of Annuloaortic Ectasia, Aortic Regurgitation, and Pectus Excavatum in Marfan Syndrome / 日本心臓血管外科学会雑誌

ABSTRACT

Marfan syndrome may include cardiovascular disease co-exising with thoracic deformities. A 24 year-old man given a diagnosis of Marfan syndrome and annuloaortic ectasia (AAE), aortic regurgitation (Ar) and pectus excavatum, was referred to our hospital due to the rapid dilatation of a root aneurysm. Chest computed tomography showed a root aneurysm measuring about 60×55 mm in diameter with mild Ar. Moreover, the sternum, which had been displaced in a posterior direction, contacted with the root aneurysm and heart. The heart was deviated to the left, because of compression from the sternum. We performed a concomitant repair of AAE and Ar and pectus excavatum with partial sternal turnover and elevation, and Bentall procedure. First, median skin incision was made, and dissected to the sternum. The ribs and cartilage below the third rib were cut, and the sternum was transected at the two-thirds point. The root aneurysm and heart were visible so it was easier to operate than a post median sternotomy. A cardiopulmonary bypass was established by ascending aortic perfusion, right atrial drainage and pulmonary arterial venting. The Bentall procedure was done using a Carrel patch methods. The removed sternum was formed flat and turned over, and sternum elevation was perfomed using sternal wire, after cutting and removing the excess ribs and costal cartilage. The postoperative course was uneventful with good hemodynamic and respiratory function. Concomitant surgery provides good operative exposure, which can avoid accidental aneurysm laceration, although operation time is longer and operative invasion and bleeding are greater than in staged operations.