Title of article :
Anomalous Coronary Artery Origin in a Young Patient with Marfan Syndrome
Author/Authors :
Duarte, S. B. C. P. Heart Institute (InCor) - University of Sao Paulo Medical School, Brazil , Beraldo, D. O. Hospital Renascentista, Brazil
Abstract :
Marfan syndrome is an autosomal dominant genetic disorder that a0ects connective tissue and is caused by mutations in the
1brillin 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 insu8ciency, it increases risk of acute aortic dissection and death.
Coronary artery anomalies a0ect 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. )e 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 1stulas, 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.
Keywords :
Coronary Artery Origin , Marfan Syndrome
Journal title :
Case Reports in Cardiology