An asymptomatic 12-year-old boy with Marfan syndrome presented for routine follow-up and to establish cardiology care at our institution. His previous echocardiograms were reportedly normal. An electrocardiogram showed normal results. A routine echocardiogram revealed an ectatic proximal right coronary artery. Subsequent coronary computed tomographic angiograms showed a fusiform aneurysm beginning at the right coronary artery ostium (Fig 1). The aneurysm was 8 mm long and 5.2 cm in diameter (Z score, +3.8). The patient was prescribed low-dose aspirin.
Citation: Texas Heart Institute Journal 48, 4; 10.14503/THIJ-18-6932
Comment
Marfan syndrome is an autosomal dominant genetic disease caused by a mutation in the fibrillin FBN1 gene (estimated prevalence, 1 in 3,000–10,000 people).1 The gene is integral to elastic connective tissue, so patients with Marfan syndrome often have related abnormalities, especially cardiovascular, skeletal, and ocular.2 Chief among the cardiovascular complications is aortic root dilation, which carries the risk of aortic dissection and may suggest a need for prophylactic aortic root replacement. In Marfan syndrome, coronary artery involvement is usually a consequence of aortic dissection, and isolated coronary artery aneurysms are rare3; to our knowledge, ours is only the second reported case in a pediatric patient.4
Routine coronary monitoring with the use of echocardiography and other imaging methods is important in patients with Marfan syndrome, including children, because patients who have coronary aneurysms are often asymptomatic.5 Detecting an aneurysm will enable early treatment and possibly improve the patient's overall prognosis.
Section editor: Raymond F. Stainback, MD
Computed tomographic angiograms. A) Curved multiplanar reformatted image shows a mildly dilated fusiform aneurysm (arrow) beginning at the right coronary artery ostium. B) Three-dimensional volume-rendered image shows the 8-mm-long, 5.2-mm-diameter aneurysm (arrow).
Contributor Notes
