Giant coronary aneurysm in a patient with familial aortic aneurysm/dissection: Medial degeneration extending to coronary artery

SummaryFamilial thoracic aortic aneurysms and dissections occur as part of known syndromes such as Marfan syndrome, but can also be inherited in families in an autosomal dominant manner as an isolated condition. However, involvement of coronary artery aneurysm/dissections is rare in these patient subsets. A 37-year-old male, normal in skeletal appearance, with a distinct family history of aortic dissection, underwent four major cardiovascular surgeries since age 30 due to giant coronary aneurysm and coronary stenosis, abdominal aortic dissection, and Stanford type A aortic dissection. Pathological examinations demonstrated the left main coronary aneurysm was composed of degenerated, lacerated media, with findings of decreased elastic fibers, deposition of mucopolysaccharide, and cystic medial necrosis. These pathological features found in the coronary aneurysm were identical to those of the aortic wall and radial artery segments in this patient. This case suggests that initial coronary evaluation may be warranted in some families to optimize the clinical management of patients affected with this disease.