ABSTRACT
The authors present the clinical history of a patient with Marfan's syndrome (MS) and aortic insufficiency, who expired at 22 years of age, in order to illustrate the clinical evolution and review the management of this problem. It is known that patients with MS and heart failure due to dilatation of the aortic annulus have an early mortality of almost 100%. The authors conclude that echocardiographic follow up of these patients is vital in their medical and surgical management and that the use of beta blockers is the best medical therapy, and that prophylactic surgery with composite graft (aortic valve and ascending aorta) gives the best survival rate.
Subject(s)
Cardiovascular Diseases/diagnosis , Marfan Syndrome/diagnosis , Adult , Aortic Valve/diagnostic imaging , Aortic Valve/pathology , Aortic Valve Insufficiency/diagnosis , Aortic Valve Insufficiency/etiology , Aortic Valve Insufficiency/pathology , Cardiovascular Diseases/etiology , Cardiovascular Diseases/pathology , Echocardiography , Electrocardiography , Humans , Male , Marfan Syndrome/complications , Marfan Syndrome/pathologyABSTRACT
The authors present the clinical history of a patient with Marfan's syndrome (MS) and aortic insufficiency, who expired at 22 years of age, in order to illustrate the clinical evolution and review the management of this problem. It is known that patients with MS and heart failure due to dilatation of the aortic annulus have an early mortality of almost 100%. The authors conclude that echocardiographic follow up of these patients is vital in their medical and surgical management and that the use of beta blockers is the best medical therapy, and that prophylactic surgery with composite graft (aortic valve and ascending aorta) gives the best survival rate
