[Nebulized prostacyclin treatment for pulmonary hypertension in a 2-year-old girl].

INTRODUCTION

Primary pulmonary hypertension is an unusual disease of unknown aetiology in childhood. It is defined as a mean pulmonary artery pressure greater than 25 mmHg at rest with normal wedge pulmonary pressure and absence of secondary causes. Treatment is based on diuretics and calcium-channel blockers and, in non-responders, on continuous intravenous prostacyclin. We report the case of a patient who was treated with nebulized prostacyclin who showed clinical improvement for 3 months.

CASE REPORT

A 2-year-old child with right heart failure was admitted and stabilized with oxygen, restriction of liquids, and diuretics. The patient was diagnosed with primary pulmonary hypertension after exclusion of secondary causes. Cardiac catheterization confirmed the diagnosis. Pulmonary vascular reactivity did not test positive. Because of clinical deterioration, treatment with nebulized prostacyclin was started. We observed clinical improvement with a decrease in oxygen and diuretics demand for 3 months. After this period, the patient deteriorated and continuous intravenous prostacyclin was started. The patient died of heart failure 6 months after diagnosis.

DISCUSSION

The use of prostacyclin in the management of primary pulmonary hypertension is an alternative in non-responding patients. Clinical improvement and decrease in oxygen demand support the use of nebulized prostacyclin as a useful approach in pediatric patients.