Giant coronary artery aneurysm associated with familial retinal artery macroaneurysm: a case report

The term 'Giant' coronary aneurysm is usually defined as any coronary aneurysm more than 8 mm in maximum diameter. The form of familial retinal arterial macroaneurysms (FRAMs) is a rare autosomal recessive disease that is described by arterial aneurysm formation in the retina. Here, we report an association of coronary artery aneurysms with FRAM in a young male who presented with the acute coronary syndrome.

A 31-year-old male smoker presented to the emergency department with atypical chest pain for 5 days. Blood investigations showed raised troponin enzymes. Review of his past medical history revealed decreased vision in the left eye, starting at the age of 10 years which progressed to blindness. He was diagnosed to have IGFBP7 mutation which causes eye manifestations in the form of FRAM. Fundoscopy showed bleeding retinal artery macroaneurysms in the right retina and sub-retinal gliosis suggesting laser treatment for the prior retinal arterial aneurysm. Coronary angiogram revealed a large aneurysm in the proximal segment of the left anterior descending (LAD) artery. Cardiac computed tomography scan with contrast was done which showed a 2.28 × 1.64 cm coronary aneurysm at the proximal segment of the LAD artery with peripheral calcification with a narrow neck of about 0.6 cm. After the heart team discussion, he underwent surgical ligation of the LAD and coronary aneurysm with implantation of the saphenous venous graft to distal LAD.

Coronary artery aneurysms can be a part of multisystem diseases like FRAM. The management should be individualized based on symptoms at presentation, size of coronary aneurysms, and local expertise.