[Retinal diseases with primary pulmonary hypertension]

Primary pulmonary hypertension (PPH) is a rare but fatal disease that results in right heart failure from idiopathic occlusion of the pulmonary arteries. Patients develop stagnation of venous pressure. PPH occurs most commonly in young and middle-aged women and the mean survival time from onset of symptoms is 2-3 years. The aetiology of PPH is unknown, although familial occurrence of the disease suggests a genetic predisposition. Pulmonary transplantation is reserved for patients who fail medical therapy. Pathophysiologically, PPH is a functional and structural alteration of the pulmonary blood vessels.

The authors retrospectively reviewed the clinical records of three patients (mean age 34 years) diagnosed with PPH and retinal diseases.

All three patients developed central retinal vein occlusion (CRVO). One patient developed a neovascular glaucoma and on the other eye a serous retinal detachment with uveal effusion. A few months after the central vein obstruction was diagnosed two patients died as a result of PPH.

The elevated venous pressure found in PPH is responsible for decreased choroidal perfusion and reduced venous blood outflow from the eye. This would explain the clinical findings of serous retinal detachment and central retinal vein occlusion in these patients.