Current Diagnosis and Prescription for the Marian Syndrome: Aortic Root and Valve Replacement

Since 1976, the procedure of choice for patients with Marian aneurysm of the ascending aorta at this institution has been the Bentall procedure or its modification. One hundred seventy-four Marian patients have had ascending aortic aneurysm repair with a prosthetic composite graft. An additional four children and two adults have had aortic root replacement with a cry preserved homograft. Thirty-seven (21%) patients came to operation with an acute or chronic dissection involving the ascending aorta. Thirty-one (17%) patients having aortic root replacement also had mitral valve repair or replacement. The first 86 patients in the series had a hemostatic Bentall wrap constructed; however, in the last 88 patients, the ascending aorta has been transected completely and the aortic wall tacked loosely over the composite graft. It is our preference to construct a direct end-to-side anastomosis of each coronary ostium to the side of the composite graft. In the past, we used the Cabrol interposed graft for low-lying coronary ostia, but currently favor the “button technique” for this procedure. One hundred fifty-six of 180 patients underwent elective root replacement with no hospital mortality. Twenty-four patients underwent urgent or emergency surgery; 17 of these patients with acute dissection or rupture underwent emergency surgery. Two of these patients with rupture died intraoperatively. Therefore, overall hospital mortality among the 180 patients was 1.1%. There have been 14 late deaths among 178 patients discharged from the hospital (7.9%). Five of these late deaths occurred among our first 11 patients operated on between 1976 and 1979. Since 1980, there have been nine late deaths among 167 patients (5.4%). Three of these nine late deaths were related to late dissection of the distal unoperated thoracic or abdominal aorta. Satisfactory results can be achieved in patients with Marian aneurysms using the Bentall operative repair, and long-term results have been improved further by elimination of the aortic wrap. Close follow-up of the thoracic and abdominal aorta for late dilatation and dissection remains an essential part of the management of Marian patients. (J Card Surg 1994;9Suppl:177–781)