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Vol. 53. Issue 4.
Pages 372-376 (1 October 2000)
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Vol. 53. Issue 4.
Pages 372-376 (1 October 2000)
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Prostaciclina nebulizada como tratamiento de la hipertensión pulmonar primaria en una niña de 2 años
Nebulized prostacyclin treatment for pulmonary hypertension in a 2-year-old girl
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R. Puigarnau Vallhonrat*, M. Pons Ódena, E. Carballo Ruano
Unitat Integrada Hospital Sant Joan de Déu-Hospital Clínic. Servicio de Pediatría. Sección de Cardiología. Universidad de Barcelona. Esplugues de Llobregat. Barcelona
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Introducción

La hipertensión pulmonar primaria es una entidad de origen desconocido e infrecuente en la edad pediátrica. Se define como un aumento de la presión media de la arteria pulmonar superior a 25 mmHg en reposo con presión de enclavamiento normal y ausencia de causas secundarias. En el tratamiento se usan los diuréticos, bloqueadores del canal del calcio y, en los pacientes que no responden, prostaciclina endovenosa continua. Presentamos una paciente tratada con prostaciclina nebulizada objetivándose una mejoría clínica durante 4 meses

Observación clínica

Niña de 2 años de edad ingresada por presentar fallo cardíaco derecho que se estabiliza con oxigenoterapia, restricción de líquidos y diuréticos. La paciente es diagnosticada de hipertensión pulmonar primaria tras excluirse las etiologías secundarias. Se realiza cateterismo cardíaco confirmatorio del diagnóstico, sin respuesta positive al test de vasodilatación. Dado el deterioro clínico se inicia tratamiento con prostaciclina nebulizada. Se observa una mejoría clínica y una disminución de las necesidades de oxígeno y diuréticos durante 4 meses. Tras este período presenta un empeoramiento clínico, por lo que se decide instaurar tratamiento con PGI2 endovenosa continua. La paciente fallece a los 6 meses del diagnóstico por fallo cardíaco

Discusión

El uso de prostaciclina en el manejo de la hipertensión pulmonar primaria es una alternativa aceptada en aquellos pacientes que no responden al tratamiento habitual. La mejoría transitoria en la calidad de vida y las necesidades de oxígeno en nuestra paciente apoya el uso de prostaciclina nebulizada como opción útil en pacientes pediátricos.

Palabras clave:
Hipertensión pulmonar primaria
Niños
Prostaciclina nebulizada
Prostaciclina infusión continua
Primary pulmonary hypertension
Children
Nebulized prostacyclin
Continuous intravenous prostacyclin
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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Copyright © 2002. Asociación Española de Pediatría
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