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

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          Abstract

          Background

          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.

          Case summary

          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.

          Discussion

          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.

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          Most cited references15

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          Coronary artery aneurysm: a review.

          Coronary artery ectasia (CAE) is found in 0.3-5% of patients undergoing coronary angiography. Atherosclerosis is the main cause, followed by Kawasaki disease and infectious emboli. The exact pathogenesis has not been diagnosed as yet, but an inflammatory process is underlying. Symptoms, if present, are usually related to myocardial ischemia. Angiography is the mainstay for diagnosis. The prognosis is generally favorable. Thromboembolic complications are rare with antiplatelet therapy, and spontaneous rupture generally is rare but occurs more commonly in Kawasaki disease. Management varies from antithrombotic therapy to surgical ligation. Controlling coronary heart disease risk factors sharply affects the prognosis in patients with CAE.
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            Coronary artery aneurysm: a review and hypothesis regarding etiology.

            Coronary artery aneurysm is an uncommon condition that can be a cause of death when it thromboses or ruptures. It is always associated with destruction of the tunica media, usually associated with atherosclerosis, and commonly associated with chronic inflammation. To review the pathology, epidemiologic and clinical features, and pathophysiology of coronary artery aneurysm, particularly new research results, drawing out implications for the understanding, diagnosis, and treatment of this condition. Pertinent literature and illustrative cases at our institution. Inflammation spilling over into the tunica media from the tunica intima may link atherosclerosis to aneurysm formation, but vasculitis without atherosclerosis causes coronary artery aneurysms in young children with Kawasaki disease. Increased proteolysis of extracellular matrix proteins is probably one mechanism of coronary artery aneurysm formation, either due to overactive matrix metalloproteinases or underactive inhibition of these proteinases, and an excess of transforming growth factor beta may be another mechanism in the pathogenesis. Coronary atherosclerosis is a universal disease of adults, but only 1.5% of them have coronary aneurysms; this small group may be those with a second coronary artery disease, such as vasculitis.
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              Aneurysmal coronary artery disease: An overview

              Aneurysmal coronary artery disease (ACAD) comprises both coronary artery aneurysms (CAA) and coronary artery ectasia (CAE). The reported prevalence of ACAD varies widely from 0.2 to 10%, with male predominance and a predilection for the right coronary artery (RCA). Atherosclerosis is the commonest cause of ACAD in adults, while Kawasaki disease is the commonest cause in children and adolescents, as well as in the Far East. Most patients are asymptomatic, but when symptoms do exist, they are usually related to myocardial ischemia. Coronary angiography is the mainstay of diagnosis, but follow up is best achieved using noninvasive imaging that does not involve exposure to radiation. The optimal management strategy in patients with ACAD remains controversial. Medical therapy is indicated for the vast majority of patients and includes antiplatelets and/or anticoagulants. Covered stents effectively limit further expansion of the affected coronary segments. Surgical ligation, resection, and coronary artery bypass grafting are appropriate for large lesions and for associated obstructive coronary artery disease.
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                Author and article information

                Contributors
                Role: Handling Editor
                Role: Editor
                Role: Editor
                Role: Editor
                Role: Editor
                Journal
                Eur Heart J Case Rep
                Eur Heart J Case Rep
                ehjcr
                European Heart Journal: Case Reports
                Oxford University Press
                2514-2119
                February 2022
                09 February 2022
                09 February 2022
                : 6
                : 2
                : ytac057
                Affiliations
                King Fahad Medical City , Dabab Street, Sulaimaniya, PO Box 221124, 11311 Riyadh, Saudi Arabia
                Author notes
                Corresponding author. Tel: +966 539417315, Email: azamshah165@ 123456hotmail.com
                Author information
                https://orcid.org/0000-0003-2451-413X
                https://orcid.org/0000-0002-0120-3681
                Article
                ytac057
                10.1093/ehjcr/ytac057
                8922714
                35299703
                e4119e11-d644-4b2f-afc2-2950769bbfe5
                © The Author(s) 2022. Published by Oxford University Press on behalf of the European Society of Cardiology.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License ( https://creativecommons.org/licenses/by-nc/4.0/), which permits non-commercial re-use, distribution, and reproduction in any medium, provided the original work is properly cited. For commercial re-use, please contact journals.permissions@oup.com

                History
                : 14 September 2021
                : 19 October 2021
                : 19 January 2022
                : 25 February 2022
                Page count
                Pages: 6
                Categories
                Case Report
                Ehjcr/1
                Ehjcr/2
                Ehjcr/4
                AcademicSubjects/MED00200

                case report,coronary artery aneurysm,acute coronary syndrome,familial retinal arterial macroaneurysms (fram)

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