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 Table of Contents  
Year : 2022  |  Volume : 2  |  Issue : 2  |  Page : 577-578

Severe rheumatic heart disease presenting as central retinal artery occlusion: A case report

Department of Ophthalmology, Sheikh Shakbout Medical City, Abu Dhabi, United Arab Emirates

Date of Submission25-Aug-2021
Date of Acceptance18-Oct-2021
Date of Web Publication13-Apr-2022

Correspondence Address:
Rawdha Al Nuaimi
Department of Ophthalmology, Sheikh Shakbout Medical City, Abu Dhabi
United Arab Emirates
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_2182_21

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Keywords: Central retinal artery occlusion, emboli, hyperlipidaemia, rheumatic heart disease

How to cite this article:
Al Nuaimi R, Srinivasulu A, Al Marzouqi S. Severe rheumatic heart disease presenting as central retinal artery occlusion: A case report. Indian J Ophthalmol Case Rep 2022;2:577-8

How to cite this URL:
Al Nuaimi R, Srinivasulu A, Al Marzouqi S. Severe rheumatic heart disease presenting as central retinal artery occlusion: A case report. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 May 26];2:577-8. Available from: https://www.ijoreports.in/text.asp?2022/2/2/577/342933

Retinal artery occlusion is a rare first presentation of calcified aortic stenosis and valvular heart diseases. The emboli may be recurrent, potentially bilateral, and may cause devastating complications. Early diagnosis and management are very crucial to save the patient's life and vision. A 37-year-old gentleman, working as a technical engineer, presented with sudden loss of vision in the left eye. He denies any symptoms of amaurosis fugax or any other relevant ocular history. He denies any cardiovascular symptoms such as dyspnea, orthopnea, chest pain, or syncope. There was no history of rheumatic fever and was not a known diabetic or hypertensive. Social history revealed that he was an ex-smoker with a 20-pack year history. He initially presented with a visual acuity of light perception and a relative afferent pupillary defect. Anterior segment examination was otherwise normal. Intraocular pressure was 12 mmHg. Dilated fundus examination revealed central retinal artery occlusion involving primarily inferior half of retina with an occluded cilioretinal artery. Urgent computed tomography (CT) scan of the brain without contrast was done to rule out infraction. CT angiography of the head and neck showed no evidence of narrowing or stenosis. Blood investigations such as total blood count were within normal limits, total cholesterol 6.29 mmol/L, HDL 1.34, LDL 3.74, and Hba1c 4.69. Coagulation screening shows PT 12.80 s, INR 1.03, and APTT 32.5 s. Ancillary lab investigations include ANA negative, c-ANCA, and p-ANCA normal. His Doppler scan and transoesophageal echocardiography confirmed the diagnosis of rheumatic valvular heart disease with mild to moderate aortic stenosis, moderate to severe aortic regurgitation, and moderate mitral stenosis. Autofluorescence revealed a calcific embolus at the optic disc [Figure 1]. Fundus fluorescein angiogram showed delay in arterial filling of about 2.5 min, more significantly in the inferior hemicentral artery main branch [Figure 2]. Immediate intervention with Aspirin 100 mg and intravenous acetazolamide 500 mg was done. Within 24 h, the patient had improvement in vision from light perception to counting fingers close to face. The calcified embolus that was not seen in the first day of presentation was clearly evident due to dislodgement following the reduction in intraocular pressure after the administration of the above medication. The embolus was dislodged from the preliminary portion of central retinal artery into the optic disc's surface, which led to improvement of vision. The patient was reviewed and assessed by the cardiology and hematology clinics. Due to the findings of the cardiac echocardiogram, he was placed on warfarin. He is currently under the care of valvular heart disease clinic and is planned for cardiac surgical intervention.
Figure 1: Colored fundus photo before and after IV acetazolamide

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Figure 2: (a) Autofluorescent. (b and c) Fluorescein fundus angiography

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  Discussion Top

Our literature review revealed that only a few sporadic cases have been reported, but our case is unusual because he is young and asymptomatic. Furthermore, the embolus was the first presentation and only symptom of the rheumatic valvular disease which involved multiple valves: aortic stenosis and regurgitation and mitral stenosis with enlarged left atrium. Most reported cases were in patients above the age of 50 (1). In a retrospective study by Ramakrishna et al. (2), it was reported that calcific retinal embolism may be a presenting feature of clinically undiagnosed cardiovascular disease otherwise asymptomatic patients. The reported patients had similar features to our patient and some underwent surgical cardiac intervention and were placed on an anticoagulant. Although a direct relationship has not been established between calcific retinal embolism and cardiovascular disease, it is an important factor to rule out in patients who are asymptomatic. Central retinal embolus can be a devastating complication of calcific aortic stenosis and regurgitation and mitral stenosis. However, it is rare for retinal embolization to be the presenting feature for valvular disease. It is very important to recognize it as an initial presentation of cardiovascular disease. Furthermore, acute management and further investigations can help improve the patient's vision and life such as in our case. Our patient will undergo cardiovascular surgery for aortic valve replacement. Our case is unique because our patient presented with a hemicentral retinal occlusion as a first presentation for rheumatic valvular heart disease with involvement of many valves of which are moderate aortic stenosis, severe aortic regurgitation, and moderate mitral valve stenosis in an otherwise asymptomatic young healthy individual.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.


  [Figure 1], [Figure 2]


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