Operative management of Marfan syndrome: The Johns Hopkins experience

Background. Doctor Antoine Marfan described the first case of Marfan syndro me in 1896. It was over 50 years later that the development of aortic aneur ysms and subsequent rupture was appreciated as the most life-threatening co mponent of the syndrome. Methods. Doctor Vincent Gott, at our institution, performed the first Benta ll procedure for an aneurysm of the ascending aorta in 1976. Since that tim e, the aortic root has been replaced in 231 Marfan patients. Of this group, 218 patients had a composite graft repair, 11 had an aortic root replaceme nt with a homograft, and 2 patients had valve sparing procedures. There wer e 168 males and 63 females. Of the total 231 patients, 150 were operated on by Dr Gott. The remaining 81 patients were operated on by 10 other Hopkins surgeons. The average diameter of the ascending aorta was 6.8 cm, with a r ange from 4.5 to 10. The average aortic diameter of 43 patients who had an ascending aortic dissection was 7.3 cm. Fourteen of these patients had diss ection with an aortic diameter of 6.5 cm or less. Results. Among the 198 patients who underwent elective repair, there was no 30-day mortality. Thirty-three patients underwent urgent repair with 2 dea ths, yielding a 30-day mortality of 6.1%. The mortality for the entire grou p of patients was 0.9%. Complications associated with this series of patien ts included 8 with endocarditis, 7 with thromboembolism, and 4 late coronar y dehiscences. Actuarial survival was 88% at 5 years, 81% at 10 years, and 75% at 20 years. Multivariate analysis revealed New York Heart Association classification, male gender and urgent surgery as independent risk factors for mortality. Conclusion. Marfan patients with aortic aneurysms can undergo elective surg ery with a low operative risk and excellent long-term survival with low mor bidity. We feel that elective resection of an aneurysm in a Marfan patient should occur when it approaches a diameter of 5.5 cm. It is essential that a timely diagnosis be made in this group of young patients. (C) 1999 by The Society of Thoracic Surgeons.