• Primary pulmonary hypertension

We reported a case of a 30-year-old Hispanic patient with a history of idiopathic pulmonary arterial hypertension (PAH). A baseline catheterisation showed a mean pulmonary artery pressure (PAP) of 58 mm Hg, capillary wedge pressure of 14 mm Hg, cardiac index of 2.7 l/min/m² and pulmonary vascular resistance of 7.5 WU, with no response to adenosine. A pulmonary CT scan ruled out thromboembolism or significant abnormalities (such as glass opacities, septal lines or mediastinal node enlargement commonly seen in venocclusive disease1); albumin macroaggregate lung perfusion scan showed normal perfusion without significant intrapulmonary shunt (IPS). He was started on diuretics, oxygen and sildenafil 25 mg three times a day. Despite treatment, dyspnoea worsened and 2 months later the patient was referred …