Retinal Vascular Patterns in Adults with Cyanotic Congenital Heart Disease

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Introduction: The purpose of this report is to describe retinal findings in adults with cyanotic congenital heart disease.Methods: Four patients, 27 to 47 years of age, with cyanotic congenital heart disease were enrolled from the Outpatient Clinic of the Ahmanson/UCLA Adult Congenital Heart Disease Center. Each patient had a medical history and physical examination by a cardiologist, chest X-ray, electrocardiogram, echocardiogram, complete blood count, and arterial oxygen saturation. In addition, each patient had an ocular examination by a retina specialist, color fundus photography, and fluorescein angiography.Results: The chest X-ray, electrocardiogram, and echocardiogram were diagnostic of cyanotic congenital heart disease (Eisenmenger Syndrome). Mean systemic arterial oxygen saturation was 77% (range 71–81%) and mean hematocrit was 64.5% (range 53.7–69.5%). Eight out of eight eyes had evidence of increased retinal vascular tortuosity, but no patient had ocular symptoms, and all eyes had good visual acuity.Conclusions: Increased retinal vascular tortuosity appears to be prevalent in adults with cyanotic congenital heart disease and is likely to be in response to hypoxemia and erythrocytosis. This conclusion is consistent with normalization of the retinal vascularity patterns after surgical relief of cyanosis resulting in resolution of hypoxemia and erythrocytosis.