Author(s)

Adrian Espejel-Guzman¹, Valente Fernandez-Badillo¹, Javier Serrano-Roman¹, Enrique Solorzano-Pinot¹, Sergio Criales-Vera², Maria Del Carmen Lopez-Rodriguez³, Candace Keirns⁴, Daniel Zagal-Cardoso¹, Nilda Espinola-Zavaleta^1,5*

¹Department of Nuclear Cardiology, National Institute of Cardiology Ignacio Chavez, Mexico City, Mexico.
²Department of Computed Tomography, National Institute of Cardiology Ignacio Chavez, Mexico City, Mexico.
³Department of Clinical Cardiology, National Institute of Cardiology Ignacio Chavez, Mexico City, Mexico.
⁴Shelby County Health Department, Memphis, TN, USA.
⁵Department of Echocardiography, ABC Medical Center I.A.P., Mexico City, Mexico.

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.

Marfan-Syndrome, Aortic-Dissection, Heart-Failure

Espejel-Guzman, A. , Fernandez-Badillo, V. , Serrano-Roman, J. , Solorzano-Pinot, E. , Criales-Vera, S. , Lopez-Rodriguez, M. , Keirns, C. , Zagal-Cardoso, D. and Espinola-Zavaleta, N. (2022) An Unusual Presentation of Aortic Dissection and Aneurysmatic Disease in Marfan Syndrome: A Case Report. World Journal of Cardiovascular Diseases, 12, 297-303. doi: 10.4236/wjcd.2022.126029.