Cardiac operations in children with Marfan's syndrome: indications and results

The development of new screening techniques for the early detection of Marfan's syndrome has prompted evaluation of the results of cardiac operations in children with this syndrome. The purpose of this study was to determine the surgical indications, operative results, and need for reoperation in children with Marfan's syndrome.

From 1980 to 1996, 245 patients underwent cardiac operations for complications of Marfan's syndrome; 26 (11%) were less than 18 years of age. The mean age at the time of operation was 10.3 +/- 1 years (range, 8 months to 17 years); 18 of the patients were male. Indications for operation were aortic root dilatation (15 patients), mitral regurgitation (4 patients), aortic root dilatation and mitral regurgitation (6 patients), and aortic arch aneurysm (1 patient). Operations included aortic root replacement (15 patients), aortic root replacement and mitral repair (5 patients), aortic root replacement and mitral replacement (1 patient), mitral repair (3 patients), mitral replacement (1 patient), and arch aneurysm repair (1 patient). The mean aortic root diameter in patients undergoing aortic root replacement was 6.2 +/- 0.2 cm. Only 1 patient underwent ascending aortic dissection. RESULTS. There were no operative deaths. At a mean follow-up of 67.1 +/- 10.2 months, 8 patients required a second cardiac procedure (41% +/- 17% 10-year freedom from reoperation). Indications for further operations were distal aortic pathology (3 patients), aortic root dilatation after initial mitral operation (3 patients), failed mitral repair (1 patient), and homograft degeneration (1 patient). Risk factors for a second cardiac procedure were age less than 10 years at the time of the first operation (p < 0.003) and mitral regurgitation (p < 0.04). Overall, 25 (96%) of 26 patients have undergone aortic root replacement and 11 (42%) patients have undergone a mitral procedure. There have been 4 late deaths, all of presumed cardiac origin. The 10-year survival rate is 79% +/- 10%. All surviving patients are in New York Heart Association functional class I or II.

We conclude that (1) aortic root dilatation is the most common surgical indication in children with Marfan's syndrome, (2) mitral regurgitation is the second most common indication, (3) aortic dissection is unusual in children with Marfan's syndrome, and (4) careful follow-up is necessary, particularly in younger children, because more than half of all children with Marfan's syndrome require repeated cardiac operations within 10 years.