SURGICAL-MANAGEMENT OF MARFAN-SYNDROME IN CHILDREN

Between August 1983 and January 1991, seven patients with Marfan syndr ome underwent surgery for severe cardiovascular complications. The mea n age at presentation was 5.7 months (range 4 to 9 months) in the infa nt group (n = 3), and 13.3 years (range 10 to 16 years) in a group of older children (n = 4). The primary indications for surgery in the inf ant group (performed at a mean of 3 years after diagnosis) were ascend ing aortic aneurysm with valvar regurgitation in one patient, and seve re mitral valve prolapse with regurgitation in two. In the older group , surgical indications (performed at a mean of 2.8 years after diagnos is) were ascending aortic aneurysm with valvar regurgitation in three patients and acute aortic dissection in one. For aortic surgery, a com posite valved conduit was used in four patients, and an aortic homogra ft in one. For mitral valve surgery, mechanical prostheses were used. All patients survived the primary operation. Over a mean follow-up of 17.5 patient-years (range 1 to 9 years), two patients in the infant Ma rfan group went on to further successful surgery (prosthetic mitral va lve replacement and aortic root repair with aortic homograft) at a mea n interval of 4.3 years after the initial surgery. Our results suggest that the major cardiovascular risk factors of Marfan syndrome in the young, even in those diagnosed during infancy, have been favorably cha nged by surgery with an encouraging medium-term outlook. The correct t iming of surgery is aided by echocardiography.