Author + information
- Received August 9, 1988
- Revision received February 15, 1989
- Accepted March 1, 1989
- Published online August 1, 1989.
- Dominic L. Marsalese, MD,
- Douglas S. Moodie, MD, FACC∗,
- Michael Vacante, DO,
- Bruce W. Lytle, MD, FACC,
- Carl C. Gill, MD, ACC,
- Richard Sterba, MD, FACC,
- Delos M. Cosgrove, MD, FACC,
- Matthew Passalacqua, DO,
- Marlene Goormastic, MPH and
- Andrea Kovacs, BS
- ↵∗Address for reprints: Douglas S. Moodie, MD, Department of Cardiology, Cleveland Clinic Foundation, One Clinic Center, 9500 Euclid Avenue, Cleveland, Ohio 44195-5066.
A retrospective analysis was undertaken to define the natural history and long-term follow-up of a group of patients with Marfan's syndrome. Eighty-four patients were diagnosed between January 1959 and June 1987 as having Marfan's syndrome; 68% were male; their ages ranged from 2 to 67 years (mean 26.6). Sixteen patients constituted the early surgical group (those who underwent surgery before 1979; mean age 36.1 years). Nineteen patients constituted the late surgical group (surgery in 1979 or later; mean age 33.3 years). The nonsurgical group comprised 49 patients (mean age 19.3 years).
Fifty-seven percent of the patients had a diastolic murmur and 38% had cardiomegaly at presentation. Fiftyseven percent underwent cardiac catheterization, which revealed aortic root dilation (85%), aortic regurgitation (73%), aortic dissection (33%) and mitral regurgitation (36%). Thirteen of the 19 patients in the late surgical group received a composite graft repair of the ascending aorta as compared with only 2 of the 16 in the early surgical group.
Follow-up information was obtained on 81 (96%) of 84 patients; the follow-up time was 2 to 332 months (mean 99). Thirty-one of the 81 patients died at age 3 to 63 years (mean age 35 years); 87% of the known causes of death were related to the cardiovascular system. Sixty-one percent of deaths were the result of aortic dissection or rupture or sudden cardiac death. Of the 50 survivors, 98%, including all patients in the late surgical group, were in functional class I or II. Overall survival at 5, 10 and 15 years after operation was 78.4%, 57.1% and 49.5%, respectively. The presence of a diastolic murmur or cardiomegaly, or both, at presentation was associated with poor prognosis, which can be attributed to aortic insufficiency.
There was a definite improvement in survival of patients operated on in 1979 or later as compared with survival of patients in the early surgical group (87.5 % survival at 5 years versus 56%, respectively). The improved survival in the late surgical group is attributable to earlier and more extensive surgery utilizing a composite graft with reimplantation of the coronary arteries as the procedure of choice.
- Received August 9, 1988.
- Revision received February 15, 1989.
- Accepted March 1, 1989.