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Case Reports in Cardiology
Volume 2017, Article ID 3861923, 5 pages
https://doi.org/10.1155/2017/3861923
Case Report

Anomalous Coronary Artery Origin in a Young Patient with Marfan Syndrome

1Heart Institute (InCor), University of São Paulo Medical School, Enéas de Carvalho de Aguiar Avenue 44, 05403-000 São Paulo, SP, Brazil
2Hospital Renascentista, Salvador dos Santos Nora Avenue 76, 37550-000 Pouso Alegre, MG, Brazil

Correspondence should be addressed to S. B. C. P. Duarte; rb.moc.oohay@etraudahsas

Received 24 June 2017; Revised 13 September 2017; Accepted 13 November 2017; Published 21 December 2017

Academic Editor: Aiden Abidov

Copyright © 2017 S. B. C. P. Duarte et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Marfan syndrome is an autosomal dominant genetic disorder that affects connective tissue and is caused by mutations in the fibrillin 1 gene present at chromosome 15. Aortic aneurysm is its main complication, and along the dilation of the aorta root and its descending portion (60–100%), with secondary aortic insufficiency, it increases risk of acute aortic dissection and death. Coronary artery anomalies affect between 0.3% and 1.6% of the general population and are the second leading cause of sudden death in young adults, especially if the anomalous coronary passes through aorta and pulmonary artery. The anomalous origin of the left main coronary artery in the right Valsalva sinus has a prevalence of 0.02%–0.05% and is commonly related to other congenital cardiac anomalies, such as transposition of great vessels, coronary fistulas, bicuspid aortic valve, and tetralogy of Fallot. Its association with Marfan syndrome is not known, and there is no previous report in the literature. We describe here a case of a female with Marfan syndrome diagnosed with symptomatic anomalous origin of the left coronary artery in the right Valsalva sinus.