Rapid regression of primary pulmonary hypertension.

A 14 month old child presented for investigation of tachypnoea. No parenchymal lung disease was shown on chest x ray. On echocardiography there was normal intracardiac anatomy with significant pulmonary hypertension. At cardiac catheterisation the presence of primary pulmonary hypertension was confirmed, with a partial response to inhaled nitric oxide (80 ppm) and 100% oxygen. The child was referred for assessment for heart-lung transplantation while maintained on oxygen, inhaled nitric oxide, and nifedipine. Repeat cardiac catheterisation two months after presentation showed complete normalisation of the pulmonary artery pressures.