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Published on: August 4, 2021
[Nebulized prostacyclin treatment for pulmonary hypertension in a 2-year-old girl]
R Puigarnau Vallhonrat1, M Pons Odena, E Carballo Ruano
1Unitat Integrada Hospital Sant Joan de Déu-Hospital Clínic, Servicio de Pediatría, Sección de Cardiología, Universidad de Barcelona, Esplugues de Llobregat. Barcelona.
Insights
Nebulized prostacyclin offered temporary clinical improvement for a child with primary pulmonary hypertension. This approach may be a useful alternative for non-responding pediatric patients.
Area of Science:
- Pediatric Cardiology
- Pulmonary Hypertension Research
- Critical Care Medicine
Background:
- Primary pulmonary hypertension is a rare childhood disease with unknown causes.
- Standard treatments include diuretics, calcium-channel blockers, and intravenous prostacyclin for non-responders.
- Diagnosis involves excluding secondary causes and confirming elevated pulmonary artery pressure.
Observation:
- A 2-year-old child with right heart failure was diagnosed with primary pulmonary hypertension.
- Initial stabilization included oxygen, fluid restriction, and diuretics.
- Cardiac catheterization confirmed the diagnosis, with no positive pulmonary vascular reactivity.
Findings:
- Nebulized prostacyclin was administered due to clinical deterioration.
- The patient showed clinical improvement, with reduced oxygen and diuretic needs for 3 months.
- Following this period, the patient's condition worsened, necessitating intravenous prostacyclin.
Implications:
- Nebulized prostacyclin can be a beneficial alternative for pediatric patients with primary pulmonary hypertension who do not respond to conventional therapies.
- This case highlights the potential utility of inhaled prostacyclin in managing severe pediatric pulmonary hypertension.
- Further research is warranted to establish the long-term efficacy and safety of nebulized prostacyclin in this population.
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.
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