[Cardiovascular complications of Marfan's syndrome: long-term results of surgical treatment].
Surgery has changed the prognosis of the Marfan's syndrome. Cardiovascular manifestations (aneurysmal dilatation of the aortic root and its risk of dissection, aortic insufficiency, mitral insufficiency due to valvular anomalies) represent the vital risk of the disease and reduce the life expectancy in one-third of these patients during the first 32 years. Twenty seven patients (22 to 53 years of age, mean 38) surgically treated have been followed up to 14 years (mean 6.5 years). 12 had aortic valve and ascending aorta replacement with reimplantation of coronary arteries, 9 had mitral valve replacement and 6 had simultaneous correction of aortic and mitral lesions. All patients survived the operation and late mortality was 18.5% to 14 years, with a long term survival of 90% at 5 and 70% at 10 years. Echocardiographic studies permit nowadays to measure the aortic root diameter, to appreciate the left ventricular function, to reveal a mitral prolapse and to precise valvular insufficiency, in young patients with Marfan's syndrome and during family investigation. Indication of surgical repair can be stated at the right time to prevent dissection, aortic rupture or cardiac failure with excellent and durable long term results.
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D000328:Adult; D001018:Aortic Diseases / Q000150:complications / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D006331:Heart Diseases / Q000150:complications / Q000601:surgery*; D006350:Heart Valve Prosthesis; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications* / Q000601:surgery; D008875:Middle Aged; D008944:Mitral Valve Insufficiency / Q000150:complications / Q000601:surgery*; D011183:Postoperative Complications; D015996:Survival Rate
Les complications cardio-vasculaires du syndrome de Marfan: résultats à long terme du traitement chirurgical.