The distal aorta in the Marfan syndrome

Prolonged survival of patients with Marfan syndrome after aortic root replacement has led to an increased number of patients with aortic complications beyond the root. Elective replacement of the aortic root removes the most important predilection site for aneurysms, but the distal aorta remains at risk. Predictors for aortic growth and adverse events in the distal aorta include aortic diameter, aortic distensiblity, previous aortic root replacement, hypertension and aortic regurgitation. After aortic dissection, the initial false lumen diameter is an independent predictor for late aneurysm formation. Although there are a few reports of short-term success after endovascular stent grafting of the descending thoracic aorta, stent grafting in patients with Marfan syndrome is not recommended unless intervention is clearly indicated and the risk of conventional open surgical repair is deemed prohibitive. Optimal long-term outcome demands lifelong radiographic follow-up and medical treatment with (beta-blocker therapy. After aortic dissection rigorous antihypertensive medication is of utmost importance. Losartan, an angiotensin II type I receptor antagonist, might offer the first potential for primary prevention of clinical manifestations in Marfan syndrome, but the results of clinical trials have to be awaited. (Neth Heart J 2008;16:382-6.)