Aortic root surgery in Marfan syndrome.

Abstract

Marfan syndrome (MFS) is an autosomal dominant connective disease etiologically related with FBN-1 gene mutation. The altered microfibril protein structure result in characteristic cardiovascular abnormalities including aortic root dilatation, aortic root aneurysms, and aortic dissections. Aortic root aneurysms and subsequent dissection are the major causes of reduced life expectancy in MFS patients. Prophylactic aortic root replacement has improved the survival of patients with MFS. Elective root replacement carries very low mortality and morbidity. Emergency root replacement for dissection is accompanied with higher early mortality and late deaths/interventions. Both the techniques of aortic root replacement, total root replacement (TRR) with a prosthetic valved conduit, and valve-sparing root replacement (VSRR) yield excellent early and late results. Considering the low risk of prosthetic valve-related events, the improved long-term survival, and event-free survival, TRR continues to be a very effective surgical option. VSSR also seems to be a good option for the first 10 to 15years following surgery in MFS patients. The choice of procedure depends upon available expertise, patients' choice, feasibility of anticoagulation, possibility of pregnancy, and lifestyle of the patient. Aortic root morphology and state of valve cusps also affect the decision making. In recent times, personalized external aortic root support (PEARS) with a macroporous mesh sleeve has also emerged as a promising alternative to aortic root replacement. All these patients need close monitoring for whole life after surgical intervention.