Marfan syndrome: the variability and outcome of operative management

Abstract

Purpose: This study reviews the contemporary surgical outcome of 69 patients with Marfan syndrome who underwent one or more aortic operations between July 18, 1989, and April 14, 1994.

Methods: During this 5-year period, 79 operations were performed including composite valve graft replacement (n = 28) and graft replacement of the thoracoabdominal aorta (n = 29). Fifty-seven additional operations had been performed before referral, for a total of 136 aortic operations in this group. Forty-two patients (60.9%) required multiple operations; 16 patients (23.2%) underwent three or more procedures.

Results: Follow-up was complete on all patients. The 30-day and long-term survival rates were 98.7% and 96.2%, respectively. Distal aortic replacement resulted in paraparesis in only one (2.6%) of 38 patients; no patient had development of paraplegia. No patient had a stroke after the use of profound hypothermic circulatory arrest.

Conclusion: Aortic surgery prolongs survival in patients with Marfan syndrome and currently has low associated morbidity and mortality rates. Treatment often requires multiple staged operations. The variety and multiplicity of aortic manifestations in these patients demonstrate the necessity for lifelong cardiovascular surveillance to detect new or recurrent disease.