Bilateral nongranulomatous uveitis with transposition of the great arteries: A rare case report.

In this case report, we describe for the first time an association between transposition of the great arteries (TGAs), a congenital heart disease, and uveitis. We hope that this atypical finding in a patient with TGA will add to the clinical spectrum of ophthalmic conditions that can be seen with TGA. A 16-year-old boy, diagnosed and operated for TGA, presented with redness and pain in the left eye. The patient underwent complete ophthalmological and systemic examination. Detailed blood workup was done to rule out other causes of uveitis. A working diagnosis of bilateral nongranulomatous uveitis in status postopen-heart surgery was made, and the patient was successfully treated. Thus, uveitis can develop in patients with TGA, and prompt treatment can restore vision in these patients. Copyright:

Introduction

Transposition of the great arteries (TGAs) is one of the most common congenital cyanotic heart diseases and represents 5%–7% of all congenital heart diseases. It has an incidence of 20–30 per 100,000 live births, and the mortality rates range from 2.6% to 4%.[12] It is characterized by ventriculoarterial (VA) discordance, with the aorta arising from the right ventricle and the pulmonary artery from the left ventricle. Uveitis is a form of ocular inflammation involving the uveal tissue, that is, the iris, ciliary body, and choroid. It can be subdivided into granulomatous and nongranulomatous types.[3] While associations between congenital heart disease and uveitis have been described and observed in patients, to the best of our knowledge, a case of uveitis in a patient with TGA has not previously been reported. We present a rare finding of bilateral uveitis and what we believe to be the first case of a patient with uveitis of unknown etiology in the setting of TGA.

Case Report

A 16-year-old boy presented with complaints of redness and diminution of vision in the left eye (oculus sinister [OS]) for the past 8 days. He gave a history of recurrent episodes of pain and redness in both the eyes for the past 4 years. The diagnosis of TGA with a ventricular septal defect (VSD) with pulmonary stenosis (PS) had been made at the age of 7 years by cardiac echography, catheterization, and coronary angiography. Subsequently, the patient underwent Rastelli's repair successfully. A computed tomography coronary angiogram of the heart and great vessels with three-dimensional reconstruction done 2 years ago showed left outflow tract connected to the aorta and the right outflow tract communicating through the aorta homograft with the pulmonary artery [Figure 1].

Figure 1

(a and b) 64-slice computed tomography coronary angiogram of the heart and great vessels with three-dimensional reconstruction showing the anatomy of the coronary artery. (c and d) The left outflow tract is seen connected to the aorta, and the right outflow tract is seen communicating through the aorta homograft with the pulmonary artery

On physical examination, the patient had normal arterial oxygen saturation. His weight was 36 kg with normal pulse and blood pressure. On ocular examination, his best-corrected visual acuity at presentation was counting finger at 2 m in the right eye and 6/18 in the left eye. Slit-lamp examination showed quiet anterior chamber (AC) in the right eye (Oculus dextrus [OD]) without any vitreous cells and AC flare 1+ and cells 2++ were noted in the OS with 360 degree posterior synechiae [Figure 2]. The patient had posterior synechiae in OS with early cataract in both eyes (oculus utro). Fundus examination was within normal limits with the absence of vitritis in OS. Intraocular pressure was within normal range. Ultrasonography B-scan showed normal posterior segment OD. There were no oral or genital ulcers and any skin changes.

Figure 2

Slit-lamp photograph of the left eye showing anterior chamber flare with posterior synechiae and cataract formation

Blood examination revealed normal complete blood cell counts. Erythrocyte sedimentation rate was raised (24 mm at the end of 1st h, by Westergren method). Serological tests for uveitis were negative, including human leukocyte antigen (HLA) B-27, antinuclear antibody, antinuclear cytoplasmic antibody, angiotensin-converting enzyme, rheumatoid factor, and Venereal Disease Research Laboratory test. Antistreptolysin O titers and C-reactive protein levels were within normal limits.

Hence, a diagnosis of bilateral nongranulomatous uveitis in status postopen-heart surgery for congenital cyanotic heart disease (transposition of the great vessels with VSD with PS) was made, and the patient was started on topical 1% prednisolone acetate 1 hourly in tapering dose and atropine 1% eye drops. One month after the initiation of the therapy, there was the resolution of uveitis with improvement in visual acuity. He was reviewed after 1 month with vision improved to 6/36, N.12 in OS. He was asymptomatic for the next 3 years, and in his last visit, he had progression of cataract in OS and was advised cataract surgery, but he did not turn up for the procedure.

Discussion

Anomalous origin seen in the aorta arises from the right ventricle and pulmonary artery from the left ventricle. Oxygenated blood from the lungs is returned to them and desaturated blood is pumped around the systemic circulation. The pulmonary and systemic circulations are separate and in this situation are incompatible with life and mixing of blood is carried out via a ventricular septal defect, an atrial septal defect or patent ductus arteriosus.[1234] There are two subtypes of TGA: first, complete TGA (dextro-TGA or d-TGA) second, the congenitally corrected transposition TGA (levo-TGA or l-TGA). The survival of these patients depends on the presence of communication or shunt between the two circulations. This shunt can be an atrial septal defect (ASD), VSD, or patent ductus arteriosus. A VSD is seen in up to 50% of patients.[4]

Ophthalmologic problems associated with congenital heart disease are rare. Reported ocular findings in patients with cyanotic heart disease are retinal vascular tortuosity, retinal hemorrhages, disk edema, retinal edema, and central retinal vein occlusion.[56]

Ocular abnormalities in a case of TGV with VSD with PS have not been described in literature before. Wu et al. reported uveitis in the case of Tetralogy of Fallot (TOF). This patient developed ischemic retinopathy with uveitis due to chronically low arterial oxygen saturation levels. Since our case had a normal oxygen saturation, ischemia could not have resulted in intraocular inflammation.[7] Literature review also showed a few case reports of infectious endocarditis (IE) with uveitis and several case reports of IE with endophthalmitis.[8]

One study reported uveitis in a case of IE with diabetes. In this case, the uveitis persisted for over 3 months.[9] In all these cases of IE, the causative organism was found to be β-hemolytic Group B streptococcus. Uveitis with poststreptococcal syndrome is an autoimmune reaction, which occurs after the infection with Group A streptococci.[10] However, in the present case, the patient was healthy, and blood cultures were negative. Furthermore, in the present study, the patient showed no manifestations, including reactive arthritis or acute glomerulonephritis. In addition, ASO titer was normal in this patient. We know that congenital heart disease can be associated with retinal vascular changes.[78] HLA-related anterior uveitis can be crossed linked with other HLA-related heart condition. However, our patient was HLA B-27 negative. Complete heart block-associated uveitis disease has been reported in the literature also.[8910] A study showed congenital rheumatic heart disease can have atypical uveitis-related problem.[910] Sometimes, atypical uveitis may be an incidental finding in heart-related disease condition.

While an association between IE, TOF, and uveitis has been described, our finding of bilateral uveitis in TGA is a rare occurrence. Furthermore, to the best of our knowledge, we are the first to report uveitis as a finding in TGA. Our patient demonstrated AC reaction with no KPs on his corneal endothelium that could not be attributed to any known cause. We hope that this atypical finding in a patient with TGA will add to the clinical spectrum of ophthalmic conditions that can be seen in association with TGA. Proper treatment can result in an early restoration of vision in such cases.

Declaration of patient consent:

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient has/have given his consent for his 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.

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Conflicts of interest

There are no conflicts of interest.