Results of surgery for aortic root aneurysm in patients with the Marfan syndrome

• Published in: European journal of cardio-thoracic surgery 2000-04, Vol.17 (4), p.415-419
• Main Authors: Tambeur, Luc, David, Tirone E., Unger, Mark, Armstrong, Sue, Ivanov, Joan, Webb, Gary
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Aged; Aneurysm, Dissecting - etiology; Aneurysm, Dissecting - mortality; Aneurysm, Dissecting - surgery; Aortic Aneurysm, Thoracic - etiology; Aortic Aneurysm, Thoracic - mortality; Aortic Aneurysm, Thoracic - surgery; Aortic Valve Insufficiency - mortality; Aortic Valve Insufficiency - surgery; Biological and medical sciences; Blood Vessel Prosthesis Implantation - mortality; Female; Humans; Male; Marfan Syndrome - complications; Marfan Syndrome - diagnosis; Medical sciences; Middle Aged; Probability; Prognosis; Retrospective Studies; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases; Surgery of the heart; Survival Analysis; Treatment Outcome
• ISSN: 1010-7940; 1873-734X
• DOI: 10.1016/S1010-7940(00)00379-1

To evaluate the results of two operations, aortic valve-sparing and aortic root replacement, in patients with aortic root aneurysm and the Marfan syndrome. A retrospective review of 78 consecutive patients with aortic root aneurysm and the Marfan syndrome according to the Gent criteria indicated that 42 patients with normal aortic cusps had an aortic valve-sparing operation, and 36 patients had aortic root replacement (mechanical valve in 25 patients and biological valve in 11). The mean age was similar in both groups, but patients who had aortic root replacement had larger aneurysms, higher grade aortic insufficiency, worse left ventricular function and more cardiac co-morbidity than patients who had aortic valve-sparing operations. The mean follow-up was 41+/-35 months for patients who had aortic valve-sparing, and 65+/-50 months for those who had aortic root replacement. Kaplan-Meyer estimates of survival at 5 years was 100% for patients who had aortic valve-sparing, and 88+/-6% for those who had aortic root replacement (P=0.04). Five patients who had aortic root replacement required seven aortic root re-replacements: three for endocarditis and four for valve failure (biological valves). There have been no reoperations in patients who had aortic valve-sparing operations and annual Doppler echocardiography revealed mild or no aortic insufficiency in 39 patients and moderate aortic insufficiency in three. These data suggest that aortic valve-sparing operations are safe in patients with the Marfan syndrome and may provide better clinical outcomes than aortic root replacement. Since the size of the aneurysm often determines the feasibility of a valve-sparing procedure, we now recommend surgery when the diameter of the aortic root reaches 50 mm in patients with the Marfan syndrome who have echocardiographically normal aortic valve cusps.