Abstract
Introduction:
Marfan syndrome (MFS) is a common heritable connective tissue disease involving multiple organs. Even though the clinical manifestations of MFS can be various, aortic root aneurysm is estimated as one of the most serious complications. We herein describe an individualized treatment decision-making process for a 23-year-old male with MFS, suffering from a giant but stable aortic root aneurysm which is extremely rare at his age.
Case:
The patient, a 23-year-old male with a family history of MFS, presented to our cardiovascular department because of progressive exertional chest distress, fatigue and occasional precordial pain. Physical examinations revealed six-foot-three inches of height, high myopia, and a diastolic murmur at the aortic valve area. Laboratory examinations for systemic vasculitis and infectious diseases were negative. The transthoracic echocardiography (TTE) and enhanced thoracic computed tomography (CT) scan revealed the existence of a giant aortic root aneurysm (125.1 mm in short-axis), severe aortic valve regurgitation, cardiac dilatation (LV; 99 mm in diastolic diameter) and a poor ejection fraction (EF; 18%). Considering the risk of rupture or dissection of the dilated aortic root, we successfully performed the Bentall procedure based on the intraoperative exploration results. Postoperative thoracic CT scan revealed a normal sized reconstructed aortic root, and the patient was discharged uneventfully 7 days later.
Conclusion
It is extremely rare to report such a giant aortic root aneurysm in a young patient. In the treatment decision-making process, the patient’s specific situation should be taken into consideration. The composite replacement of the aortic valve and ascending aorta should be performed if the patient is not suitable for valve-sparing operation.
Clinical Significance of Aortic Root Modification Associated With Bicuspid Aortic Valve in Marfan Syndrome
