[A rare cause of pulmonary arterial hypertension diagnosed in an elderly patient].

Pulmonary arterial hypertension (PAH) is a rare and incurable disease, related to right ventricle overload and failure, which are late consequences of asymptomatic progressive pulmonary vascular occlusion. The clinical approach requires: a high clinical suspicion; the detection and confirmation of PAH by echo-Doppler and right heart catheterisation; identification of an etiology; assessment of functionality and life expectancy; and reversibility testing. We present the case of a 68-year-old male patient presenting with progressive fatigability and shortness of breath, abnormal heart beats in the last 4 years, aggravated in the last year.