Early and Long-Term Results of a Valve-Sparing Operation for Marfan Syndrome

Abstract

Background —We have previously described the experience, rationale, and development of a valve preserving technique, but its role in patients with Marfan syndrome has not previously been defined. Here, we attempt to determine the early and long-term results, timing, and determinants of outcome of this operation in patients with Marfan syndrome. Methods and Results —Since 1979, 82 patients (73.2% of all patients with Marfan syndrome undergoing resection of aneurysm of the ascending aorta) were operated on using this technique. Ages ranged from 2 to 69 years (mean, 33.9 years). In all, there were 4 early deaths (4.9%), 2 with acute dissection and 2 with chronic aneurysm operated on as emergencies. There were no early deaths in 67 patients operated on electively. Actuarial survival for patients operated for chronic aneurysm was 94.2%, 94.2%, and 94.2% at 1, 5, and 10 years, respectively; that for acute dissection was 72.7%, 63.6%, and 63.6%; and that for chronic dissection was 100%, 85.7%, and 75.0%. The probability of needing reoperation was 5.7%, 17.3%, and 17.3% at 1, 5, and 10 years. There were no instances of infective endocarditis or thromboembolic complications except in 2 patients operated on early in the series who had cusp extension. At the end of the follow-up, trivial or no aortic regurgitation was demonstrated in 33.3%, mild in 45.6%, moderate in 21.1%, and severe in 0. Conclusions —Valve-sparing operations are feasible in most patients with Marfan syndrome; they are applicable to patients with both dissection and chronic aneurysm. The early and long-term results are encouraging. Results are better in the absence of dissection, and prophylactic operation is warranted in some cases.

Unusual Complication of Aortic Root Reconstruction with Sparing of the Aortic Valve: Left Ventricular Outflow Tract to Right Atrial Fistula

“…The technique of securing the graft to the aortic annulus through subannular sutures serves to prevent the development of postoperative aortic annular dilatation with subsequent aortic regurgitation most frequently observed in patients with underlying connective tissue disease. 4,5 The placement of these subannular sutures in close vicinity to the thin atrioventricular septum is likely the cause of the fistulous communication between the LV and the RA observed in the two patients presented here. This LV to RA VSD is also known as a Gerbode defect.…”