Aortic surgery in patients with Marfan syndrome: Longterm survival, morbidity and function

Background and aim of the study: The natural history of patients with Marfa n syndrome is depressing; but surgical intervention on the aorta can improv e the prognosis. Study results were analyzed with reference to long-term su rvival, morbidity and function. Methods: Seventy-four Marfan patients (51 males, 23 females; mean age 41 +/ - 14 years), underwent first-time aortic surgery between 1977 and 1998. Fol low up information regarding mortality, morbidity and functional status was obtained from patient records and by questionnaire. The mean follow up was 5 years (range: 0-19 years). Forty-seven patients (64%) had a dissection, 27 (36%) an aneurysm, and 45 (61%) patients underwent emergency operations (<4 h from arrival at hospital). In 72 patients (97%) the disease affected the ascending aorta, and implantation of a composite graft was the most fre quent operation. Results: Overall 30-day mortality was 12% (3% in elective cases, 18% in eme rgency cases, p <0.05). Emergency operations and surgery extended to the ao rtic arch were risk factors for early mortality. Overall actuarial survival was 63.4 +/- 8% at 10 years. Age was the only risk factor for late mortali ty. Seventeen patients were reoperated on due to pathologies of the remaini ng aorta (n = 12), pseudoaneurysms (n = 4) and aortic valve endocarditis (n = 1). A total of five patients had endocarditis; one patient with a homogr aft required surgery, and medical treatment was successful in the other fou r patients. Five patients had neurological thromboembolic episodes without permanent damage, and six had minor bleeding complications. Freedom from ea rly and late mortality, reoperation on the aorta and major cardiovascular e vents (endocarditis episodes, thromboembolic/hemorrhagic strokes and other major bleeding/embolic episodes) was 33.3 +/- 8.1% at 10 years. At follow u p, 98% of patients were in NYHA functional class I or II, and 80% were work ing. Conclusion: Elective aortic surgery in Marfan patients can be performed wit h good results. Close follow up of patients undergoing surgery is important . The long-term functional status of surviving patients is satisfactory.