title: Cystic medial necrosis of the aorta in patients without Marfan's syndrome: surgical outcome and long-term follow-up.
abstract: A retrospective analysis was performed to determine the surgical outcome and long-term follow-up of patients with documented cystic medial necrosis of the aorta. Ninety-three patients were diagnosed as having cystic medial necrosis at the Cleveland Clinic between July 1963 and December 1987 (72% men aged 26 to 77 years, mean 55). Patients who met the standard diagnostic criteria for Marfan's syndrome were deliberately excluded. Sixty-eight percent of the patients had a diastolic murmur and chest roentgenogram revealed a dilated aortic arch in 58% and cardiomegaly in 63%. Cardiac catheterization in 76 patients demonstrated aortic root dilation in 78%, aortic regurgitation in 72%, aortic dissection in 32% and coronary artery disease in 32%. Ninety patients underwent surgery including composite graft repair with reimplantation of the coronary arteries in 34%. Follow-up, obtained on 90 (97%) of the 93 patients, ranged in duration from 0 to 137 months (mean 29). Thirty-four of the 90 patients died (age range 30 to 75 years, mean 60). Ninety-four percent of the known causes of death were related to the cardiovascular system; 65% were the result of aortic dissection or rupture or sudden death. Ninety-six percent of survivors were in New York Heart Association functional class I or II. Overall estimated survival at 1, 3 and 5 years was 72.2%, 63.5% and 57.4%, respectively. Actuarial survival in patients who underwent composite graft reconstruction was 84% at 5 years. The presence of a diastolic murmur at initial presentation was associated with a poor prognosis (p = 0.03).(ABSTRACT TRUNCATED AT 250 WORDS)
authors.affiliation: Department of Cardiology, Cleveland Clinic Foundation, Ohio 44195-5066.
authors.forename: D L
authors.initials: DL
authors.lastname: Marsalese
authors.forename: D S
authors.initials: DS
authors.lastname: Moodie
authors.forename: B W
authors.initials: BW
authors.lastname: Lytle
authors.forename: D M
authors.initials: DM
authors.lastname: Cosgrove
authors.forename: N B
authors.initials: NB
authors.lastname: Ratliff
authors.forename: M
authors.initials: M
authors.lastname: Goormastic
authors.forename: A
authors.initials: A
authors.lastname: Kovacs
mesh_terms: D000328:Adult; D000368:Aged; D001018:Aortic Diseases / Q000401:mortality / Q000473:pathology* / Q000503:physiopathology / Q000601:surgery; D002318:Cardiovascular Diseases / Q000453:epidemiology; D002423:Cause of Death; D003560:Cysts / Q000473:pathology* / Q000601:surgery; D004630:Emergencies; D005260:Female; D005500:Follow-Up Studies; D006337:Heart Murmurs; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000175:diagnosis*; D008875:Middle Aged; D009336:Necrosis; D015995:Prevalence; D011379:Prognosis; D012189:Retrospective Studies; D016019:Survival Analysis