Retinal arteriolar macroaneurysms and central retinal vein occlusion in a patient with complete pulmonary atresia

Retinopathy in cyanotic heart disease arises due to hypoxia and polycythaemia. We report the development of central retinal vein occlusion, and two arteriolar macroaneurysms in a normotensive patient with polycythaemia secondary to complete pulmonary atresia.

A 44-year-old woman with polycythaemia secondary to complete pulmonary atresia, presented with unilateral reduced vision of hand movements in the right eye. Ocular assessment and hematological investigations were performed. Management was conservative.

Visual acuity was hand movements in the right eye, 6/5 in the left eye. Fundus assessment found right central retinal vein occlusion, and vessel tortuosity in the left eye. Hematological tests revealed an elevated red blood count, hemoglobin, heamatocrit, but normal plasma viscosity. Three months later, two arteriolar macroaneurysms developed in the left superotemporal arcade. Vision of 6/5 was retained in the left eye, hence no laser treatment was given. Subsequent follow-up revealed gradual involution of both macroaneurysms.

Multiple retinal arteriolar macroaneurysms may occur in association with cyanotic heart disease such as pulmonary atresia. They appear to involute spontaneously, hence conservative management is appropriate.