Marfan syndrome. Part 2: treatment and management of patients.
Aortic disease is the main cause of death among patients with Marfan syndrome. Before the development of open surgery, most patients died in the fourth decade of life. Improvements in surgical techniques have facilitated prophylactic surgery and have dramatically changed the life expectancy of patients with Marfan syndrome. Valve-sparing techniques are becoming the standard surgical treatment for these patients, since the operative and long-term results are comparable with those obtained with the Bentall and De Bono procedure and their theoretical advantages over the Bentall and De Bono procedure are attractive for young patients. Distal aortic complications still cause substantial morbidity in patients who have undergone surgery. On the other hand, several medical approaches have appeared as alternatives or adjuncts to the standard treatment with beta-blockers. Mouse models of the disease have shown that the angiotensin II receptor blocker losartan can rescue the phenotype. Among female patients, pregnancy deserves special consideration. Aortic dissection occurs mainly in the third trimester of gestation and in patients with dilated aortas. As aortic dissection carries a high risk of maternal mortality and fetal demise, prophylactic aortic surgery is recommended before attempting pregnancy for those women with an aortic diameter exceeding 40 mm.
Cañadas
Victoria
V
Instituto Cardiovascular, Hospital Clínico San Carlos, C/Profesor Martín Lagos, sn, 28024 Madrid, Spain. victoria_canadasgodoy@yahoo.es
Vilacosta
Isidre
I
Bruna
Isidoro
I
Fuster
Valentin
V
D000319:Adrenergic beta-Antagonists / Q000627:therapeutic use; D000818:Animals; D017545:Aortic Aneurysm, Thoracic / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D019917:Blood Vessel Prosthesis Implantation; D015331:Cohort Studies; D004195:Disease Models, Animal; D005260:Female; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications / Q000188:drug therapy / Q000401:mortality / Q000601:surgery*; D051379:Mice; D008945:Mitral Valve Prolapse / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D008991:Monitoring, Physiologic / Q000379:methods; D011247:Pregnancy; D011249:Pregnancy Complications, Cardiovascular / Q000209:etiology / Q000517:prevention & control*; D011379:Prognosis; D012084:Renin-Angiotensin System / Q000187:drug effects; D018570:Risk Assessment; D012720:Severity of Illness Index; D016019:Survival Analysis; D016896:Treatment Outcome
D000319:Adrenergic beta-Antagonists