A Rare Case of Surviving Aortic Dissection With Dominant Left Coronary in a Patient With Newly Diagnosed Marfan Syndrome

Abstract

Marfan syndrome is an autosomal dominant disease with high penetrance and marked phenotypic variation caused by a mutation in the fibrillin-1 gene (FBN1) on chromosome 15. There is known multisystem involvement of Marfan syndrome; however, cardiovascular manifestations are the main cause of morbidity and mortality in affected patients. Although the most common cardiovascular manifestation is mitral valve prolapse, it is aortic involvement that has the greatest impact on prognosis. Within the aortic pathologies, aortic dilation is quite common, occurring in 60-80% of all adults with Marfan syndrome. Aortic dilation typically occurs in the aortic root due to its higher elastic fiber content, composed of fibrillin, and the wall stress and cyclic torsion that this segment experiences during ventricular systole. Aortic regurgitation, a late manifestation, is generally secondary to aortic dilation. In the general population, right coronary dominance is seen in 70%, whereas left dominance is found in approximately 18% with the remaining population demonstrating a balanced pattern. We present a patient with no prior medical history who presented with a 3-month history of cough and worsening shortness of breath, was admitted for pneumonia, was subsequently found to have an 83 mm (8.3 cm) dilation of the ascending aorta and aortic root, and survived in part due to left dominant coronary circulation.




J Med Cases. 2017;8(9):283-286
doi: https://doi.org/10.14740/jmc2890w