title: Current diagnosis and prescription for the Marfan syndrome: aortic root and valve replacement.
abstract: Since 1976, the procedure of choice for patients with Marfan aneurysm of the ascending aorta at this institution has been the Bentall procedure or its modification. One hundred seventy-four Marfan 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 cryopreserved 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%).(ABSTRACT TRUNCATED AT 250 WORDS)
authors.affiliation: Division of Cardiac Surgery, Johns Hopkins Medical Institutions, Baltimore, Maryland 21287-4618.
authors.forename: V L
authors.initials: VL
authors.lastname: Gott
authors.forename: D E
authors.initials: DE
authors.lastname: Cameron
authors.forename: B A
authors.initials: BA
authors.lastname: Reitz
authors.forename: R E
authors.initials: RE
authors.lastname: Pyeritz
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000784:Aortic Dissection / Q000601:surgery; D001014:Aortic Aneurysm / Q000601:surgery*; D001019:Aortic Rupture / Q000601:surgery; D001021:Aortic Valve / Q000601:surgery*; D001807:Blood Vessel Prosthesis; D002648:Child; D002675:Child, Preschool; D015925:Cryopreservation; D005260:Female; D006350:Heart Valve Prosthesis; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000175:diagnosis* / Q000601:surgery*; D008875:Middle Aged; D008944:Mitral Valve Insufficiency / Q000601:surgery; D011474:Prosthesis Design; D015996:Survival Rate; D013536:Suture Techniques; D014184:Transplantation, Homologous