Treatment of Refractory Pulmonary Arterial Hypertension with Inhaled Epoprostenol in an Infant with Congenital Heart Disease

Epoprostenol is a potent arterial vasodilator, and its administration by inhalation localizes its effects to the pulmonary circulation. In this case report, we describe a 3-month-old male patient with significant refractory pulmonary hypertension after pulmonary artery banding and placement of a Blalock-Taussig shunt. This patient continued to have significant hypoxic episodes despite maximal therapy with sedation, alkalinization, sildenafil, and inhaled nitric oxide. After the addition of inhaled epoprostenol, improvements in both clinical response and echocardiography-based hemodynamics were observed. The case supports a synergistic role among the agents in the treatment of pulmonary arterial hypertension from congenital heart disease.