[Cardiovascular surgery in Marfan syndrome. A review with case examples].
It has been clearly recognized that the diagnosis, management and long-term follow-up of operated and non-operated Marfan patients require a multidisciplinary approach. Despite the high quality of medical care in this country, the diagnosis of Marfan syndrome will be delayed in a majority of the cases even if the patient has already been treated for one of the aspects of this disease. Indications for surgery have recently been simplified. Regarding aortic and mitral valve regurgitation, the indications for surgical repair are similar to those of non-Marfan patients. The aortic root and ascending aorta should be replaced when the diameter reaches 50 to 55 mm. In children and adolescents, surgery is considered indicated when the diameter of the diseased aortic segment is more than twice the diameter of the normally expected aortic arch. This review presents a single center experience in the surgical treatment of cardiovascular manifestations associated with Marfan syndrome and summarizes some interesting cases treated in the recent past. Despite the progress made in the fields of surgery, anesthesiology, intensive care and cardiology, the mortality of acute surgery because of aortic dissection remains about 10% whereas elective surgery can be performed with a perioperative risk of around 2%. Even if composite graft replacement gives excellent long-term results, some alternatives to this technique should be considered, especially in patients in whom anticoagulation with cumadines is not recommended. More recently, a Working group for Adult and Teenager Congenital Heart Disease (WATCH) has been established within the Swiss Society of Cardiology, with the aim of optimizing the management of grown-up patients with congenital heart defects. Marfan syndrome was included in the recommendations of this group. Follow-up before or after surgery is recommended at yearly (or half-yearly) intervals and should include echocardiography and/or computed tomography or magnetic resonance imaging. Finally, the patient and his family should be informed about the prognosis of the disease, the technical options in the treatment of cardiovascular manifestations, and the possibility of assistance from the Marfan Foundation.
Carrel
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Universitätsklinik für Thorax-, Herz- und Gefässchirurgie, Inselspital Bern.
D000293:Adolescent; D000328:Adult; D000784:Aortic Dissection / Q000601:surgery; D001014:Aortic Aneurysm / Q000601:surgery; D001022:Aortic Valve Insufficiency / Q000175:diagnosis / Q000601:surgery; D001807:Blood Vessel Prosthesis; D002648:Child; D006330:Heart Defects, Congenital / Q000175:diagnosis / Q000601:surgery*; D006350:Heart Valve Prosthesis; D006801:Humans; D008382:Marfan Syndrome / Q000175:diagnosis / Q000601:surgery*; D008875:Middle Aged; D008944:Mitral Valve Insufficiency / Q000175:diagnosis / Q000601:surgery