Background Neonatal persistent pulmonary hypertension (NPPH) is characterized by persistently high pulmonary vascular resistance. This condition is often secondary to chronic lung disease (CLD) with chronic pulmonary vascular changes. Although inhaled NO (iNO) therapy and high frequency oscillatory ventilation (HFOV) improved the clinical course and outcomes of many infants, pulmonary hypertension can be refractory to inhaled NO and at HFOV or the dependence of iNO therapy suggesting the need for additional approaches to severe PPHN. Case report We report a case of a girl born at 24 weeks of gestational age with a birth weight of 480gr. She was mechanical ventilated (SIMV) for two months, and after an infectious episode, she has developed oxygen-dependence and severe PPHN (PAP 125mm/Hg). This pathological condition was initially treated with HFOV (MAP 12cm H2O, FiO2 0.7% with SatO2 80%) for 25 days associated with iNO at the dose of 20ppm. Therefore, for non-clinical improvement, the girl was treated with oral Sildenafil at the dose of 2mg/kg/6h and after five days with oral Bosentan 8mg/kg/die. She has presented a significant improvement of clinical conditions 72 hours after starting treatment with sildenafil. The association of Bosentan has been decisive in improving the oxygenation and the echocardiography parameters (PAP 50mm/Hg) and for a gradual weaning from mechanical ventilation. Conclusion Many data are reported in literature about the use of Sildenafil, Bosentan and other drugs useful for the management of NPPH. However, further studies are needed to define the optimal dose, the therapeutic and side effects.

New oral therapies for neonatal persistent pulmonary hypertension in newborn with chronic lung disease

AVERSA, SALVATORE;GITTO, Eloisa;BARBERI, Ignazio
2009-01-01

Abstract

Background Neonatal persistent pulmonary hypertension (NPPH) is characterized by persistently high pulmonary vascular resistance. This condition is often secondary to chronic lung disease (CLD) with chronic pulmonary vascular changes. Although inhaled NO (iNO) therapy and high frequency oscillatory ventilation (HFOV) improved the clinical course and outcomes of many infants, pulmonary hypertension can be refractory to inhaled NO and at HFOV or the dependence of iNO therapy suggesting the need for additional approaches to severe PPHN. Case report We report a case of a girl born at 24 weeks of gestational age with a birth weight of 480gr. She was mechanical ventilated (SIMV) for two months, and after an infectious episode, she has developed oxygen-dependence and severe PPHN (PAP 125mm/Hg). This pathological condition was initially treated with HFOV (MAP 12cm H2O, FiO2 0.7% with SatO2 80%) for 25 days associated with iNO at the dose of 20ppm. Therefore, for non-clinical improvement, the girl was treated with oral Sildenafil at the dose of 2mg/kg/6h and after five days with oral Bosentan 8mg/kg/die. She has presented a significant improvement of clinical conditions 72 hours after starting treatment with sildenafil. The association of Bosentan has been decisive in improving the oxygenation and the echocardiography parameters (PAP 50mm/Hg) and for a gradual weaning from mechanical ventilation. Conclusion Many data are reported in literature about the use of Sildenafil, Bosentan and other drugs useful for the management of NPPH. However, further studies are needed to define the optimal dose, the therapeutic and side effects.
2009
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11570/1897739
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