Loeys-Dietz Syndrome Complicated by Right Coronary Artery Pseudoaneurysm

Abstract

Loeys-Dietz syndrome is a rare autosomal dominant connective tissue disorder notable for rapidly progressive vascular aneurysmal disease and craniofacial defects. Patients are at an increased risk for aneurysm rupture and dissection at younger ages compared to other aneurysmal syndromes. Early surgical intervention is important for prevention of ruptures and/or dissection. The coronary arterial tree is mostly involved as a result of postoperative complications of an aortic root repair. This fact has been sparsely reported. We report a unique case of LDS2 presenting with chest pain that was later diagnosed as a pseudoaneurysm as a result of a right coronary artery graft dehiscence.

Case report of Loeys-Dietz syndrome presenting with coronary artery aneurysm

“…6 Similarly, coronary aneurysms or pseudoaneurysms in this population have been rarely described and only diagnosed following aortic root replacement. 3 , 7 This is the first report of a patient with a TGFBR1 mutation presenting with coronary artery aneurysm without antecedent aortic replacement. This patient did have other risk factors for coronary aneurysm, including atherosclerosis and a history of significant tobacco abuse, which may have also contributed to CAA formation.…”

Coronary artery aneurysm in Loeys-Dietz syndrome: a case report

“…1 Although there are multiple reports of coronary artery dissection associated with LDS, the contribution of LDS to coronary artery aneurysm formation is undefined. 5–8 Coronary aneurysms have been reported after valve-sparing aortic root replacement, 9–12 but this is believed to be largely surgery-related and not an LDS-specific complication. 12 We believe this represents the first reported case of a spontaneous coronary artery aneurysm in association with LDS.…”