Shah Muhammad Azam, Alqahtani Abdulrahman, Alshahrani Saeed T, Alshehri Halia Zain
King Fahad Medical City, Dabab Street, Sulaimaniya, PO Box 221124, 11311 Riyadh, Saudi Arabia.
Eur Heart J Case Rep. 2022 Feb 9;6(2):ytac057. doi: 10.1093/ehjcr/ytac057. eCollection 2022 Feb.
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.
“巨大”冠状动脉瘤通常定义为最大直径超过8毫米的任何冠状动脉瘤。家族性视网膜动脉大动脉瘤(FRAM)是一种罕见的常染色体隐性疾病,其特征是视网膜中形成动脉动脉瘤。在此,我们报告一名患有急性冠状动脉综合征的年轻男性中冠状动脉瘤与FRAM的关联。
一名31岁男性吸烟者因5天的非典型胸痛就诊于急诊科。血液检查显示肌钙蛋白酶升高。回顾其既往病史发现,他从10岁开始左眼视力下降,最终失明。他被诊断出患有IGFBP7突变,该突变导致了以FRAM形式出现的眼部表现。眼底镜检查显示右视网膜有出血性视网膜动脉大动脉瘤和视网膜下胶质增生,提示之前的视网膜动脉动脉瘤已接受激光治疗。冠状动脉造影显示左前降支(LAD)动脉近端有一个大动脉瘤。进行了心脏增强计算机断层扫描,结果显示LAD动脉近端有一个2.28×1.64厘米的冠状动脉瘤,周围有钙化,瘤颈狭窄约0.6厘米。经过心脏团队讨论,他接受了LAD动脉和冠状动脉瘤的手术结扎,并植入了大隐静脉移植物至LAD动脉远端。
冠状动脉瘤可能是像FRAM这样的多系统疾病的一部分。治疗应根据就诊时的症状、冠状动脉瘤的大小和当地的专业知识进行个体化。