Njoku, P.O., Mbadiwe, N.C., Onwubere, B.J., Ejim, E.C., Anisiuba, B.C., Iyidobi, T.C., et al. (2022) Marfan Syndrome with Aortic Root Disease, Severe Heart Failure and Aortic Dissection—Two Case Reports. Nigerian Journal of Clinical Practice, 25, 205-210.
https://doi.org/10.4103/njcp.njcp_675_20

has been cited by the following article:

• TITLE: An Unusual Presentation of Aortic Dissection and Aneurysmatic Disease in Marfan Syndrome: A Case Report

  AUTHORS: Adrian Espejel-Guzman, Valente Fernandez-Badillo, Javier Serrano-Roman, Enrique Solorzano-Pinot, Sergio Criales-Vera, Maria Del Carmen Lopez-Rodriguez, Candace Keirns, Daniel Zagal-Cardoso, Nilda Espinola-Zavaleta

  KEYWORDS: Marfan-Syndrome, Aortic-Dissection, Heart-Failure

  JOURNAL NAME: World Journal of Cardiovascular Diseases, Vol.12 No.6, June 24, 2022

  ABSTRACT: Marfan’s syndrome (MFS) is an autosomal dominant disorder characterized by aortic root dilation, mitral valve prolapse and aortic regurgitation. The typical aneurysm location involves the thoracic aorta (60%). Thoracoabdominal aortic dissections are quite infrequent (10%). A type A aortic dissection can have fatal complications, such as aortic rupture and cardiac tamponade, and a prompt diagnosis is crucial. The present case involves a 46-year-old patient with Marfan’s syndrome who was admitted to the emergency department for dyspnea. The patient presented congestive signs and manifestations suggesting acute heart failure. An initial transthoracic echocardiogram (TTE)showed ventricular hypertrophy and diastolic dysfunction. Additionally, TTE showed plurivalvular regurgitation, pulmonary hypertension (42 mmHg), aortic dilatation, and a dissection flap. Coronary computed tomography angiography (CCTA) was performed, confirming the suspicion of ascending aortic dissection. The patient underwent a three-stage intervention for the repair of all the defects, with adequate recovery. This was a rare presentation of aortic dissection in MFS, which was a diagnostic challenge for the heart team.