Cardiovascular Complications In Marfan Syndrome

Cardiovascular complications of Marfan syndrome include mitral valve prolapse and regurgitation, left ventricular dilatation and cardiac failure, and pulmonary artery dilatation, but aortic root dilatation is the most common cause of morbidity and mortality. Aortic valve incompetence usually arises in the context of a dilated aortic root, and the risk of aortic rupture increases substantially when the diameter at the sinus of Valsalva exceeds 5.5 cm.5 9 Myocardial infarction may occur if an aortic root dissection occludes the coronary ostia. Marfan syndrome mortality from complications of aortic root dilatation has decreased (70% in 1972, 48% in 1995) and life

Complications Marfan Syndrome

Figure 32.4 (A) Parasagittal breath hold T1 magnetic resonance (MR) image showing pronounced dilatation of the aortic root with slight dilatation of the descending aorta in a young adult with Marfan syndrome. Line 1 represents the diameter at the sinuses of Valsalva.

(B) Parasagittal cine MR angiogram showing dilated aortic root but with normal upper ascending arch and descending aorta in a young adult.

(C) Axial computed tomographic (CT) scan at T7 of a Marfan patient showing dilated ascending and descending aorta with dissection flap anteriorly in the descending aorta and previous surgery to the ascending aorta. (D) Parasagittal reformatted CT of chest and abdomen in the same patient with contrast showing dilatation of the whole of the aorta with a spiral dissection from the arch through to the lower abdominal aorta. MR and CT images courtesy of Professor J Weir, Department of Radiology, Grampian University Hospitals NHS Trust.

Figure 32.4 (A) Parasagittal breath hold T1 magnetic resonance (MR) image showing pronounced dilatation of the aortic root with slight dilatation of the descending aorta in a young adult with Marfan syndrome. Line 1 represents the diameter at the sinuses of Valsalva.

(B) Parasagittal cine MR angiogram showing dilated aortic root but with normal upper ascending arch and descending aorta in a young adult.

(C) Axial computed tomographic (CT) scan at T7 of a Marfan patient showing dilated ascending and descending aorta with dissection flap anteriorly in the descending aorta and previous surgery to the ascending aorta. (D) Parasagittal reformatted CT of chest and abdomen in the same patient with contrast showing dilatation of the whole of the aorta with a spiral dissection from the arch through to the lower abdominal aorta. MR and CT images courtesy of Professor J Weir, Department of Radiology, Grampian University Hospitals NHS Trust.

expectancy has increased (mean (SD) age at death 32 (16) years in 1972 versus 45 (17) years in 1998),1 associated with increased medical and surgical intervention. Risk factors for aortic dissection include increased aortic diameter, extent of aortic dilatation, rate of aortic dilatation, and family history of aortic dissection.7 s

Was this article helpful?

0 0
Your Heart and Nutrition

Your Heart and Nutrition

Prevention is better than a cure. Learn how to cherish your heart by taking the necessary means to keep it pumping healthily and steadily through your life.

Get My Free Ebook


Post a comment