Ophthalmologic Diagnosis of Exacerbation of Idiopathic Pulmonary Arterial Hypertension

Abstract

Idiopathic pulmonary arterial hypertension (IPAH; formerly known as primary pulmonary hypertension) is a rare condition usually affecting young to middle-aged women in whom idiopathic obliteration of pulmonary arterioles results in increased pulmonary artery pressure and pulmonary vascular resistance, leading to right heart failure and subsequent elevation in systemic venous pressure.¹ Ocular abnormalities have rarely been reported with IPAH.^2-6 Herein, we illustrate a case in which ocular findings were the initial manifestation of clinically significant deterioration in a patient with IPAH.