RESUMO
OBJECTIVES: To describe the typical clinical course of reversible persistent pulmonary hypertension of the newborn (PPHN) from perinatal etiologies and compare that with the clinical course of PPHN due to underlying fetal developmental etiologies. STUDY DESIGN: This was a single-center, retrospective cohort study of liveborn newborns either born or transferred to our facility for higher level of care between 2015 and 2020 with gestational age ≥35 weeks and a clinical diagnosis of PPHN in the electronic health record. Newborns with complex congenital heart disease and congenital diaphragmatic hernia were excluded. Using all data available at time of collection, newborns were stratified into 2 groups by PPHN etiology - perinatal and fetal developmental causes. Primary outcomes were age at initiation, discontinuation, and total duration of extracorporeal life support, mechanical ventilation, supplemental oxygen, inhaled nitric oxide, inotropic support, and prostaglandin E1. Our secondary outcome was age at echocardiographic resolution of pulmonary hypertension. Groups were compared by t-test. Time-to-event Kaplan Meier curves described and compared (log-rank test) discontinuation of each therapy. RESULTS: Sixty-four (72%) newborns had perinatal etiologies whereas 24 (28%) had fetal developmental etiologies. The resolution of perinatal PPHN was more rapid compared with fetal developmental PPHN. By 10 days of age, more neonates were off inotropes (98% vs 29%, P < .01), decannulated from extracorporeal life support (100% vs 0%, P < .01), extubated (75% vs 37%, P < .01), and had echocardiographic resolution of PH (35% vs 7%, P = .02). CONCLUSIONS: An atypical PPHN course, characterized by persistent targeted therapies in the second week of life, warrants further work-up for fetal developmental causes.
Assuntos
Síndrome da Persistência do Padrão de Circulação Fetal , Humanos , Recém-Nascido , Estudos Retrospectivos , Síndrome da Persistência do Padrão de Circulação Fetal/terapia , Síndrome da Persistência do Padrão de Circulação Fetal/diagnóstico , Feminino , Masculino , Oxigenação por Membrana Extracorpórea , Ecocardiografia , Idade Gestacional , Respiração ArtificialAssuntos
Ventrículos do Coração/fisiopatologia , Hérnias Diafragmáticas Congênitas/complicações , Hipertensão Pulmonar/etiologia , Disfunção Ventricular Esquerda/etiologia , Ecocardiografia , Feto , Hérnias Diafragmáticas Congênitas/terapia , Humanos , Hipertensão Pulmonar/terapia , Recém-Nascido , Disfunção Ventricular Esquerda/terapiaAssuntos
Fatores Relaxantes Dependentes do Endotélio/uso terapêutico , Hipertensão Pulmonar/tratamento farmacológico , Doenças do Prematuro/tratamento farmacológico , Óxido Nítrico/uso terapêutico , Administração por Inalação , Displasia Broncopulmonar/prevenção & controle , Humanos , Recém-Nascido , Recém-Nascido Prematuro , Índice de Gravidade de DoençaRESUMO
We describe 2 infants with congenital diaphragmatic hernia with severe pulmonary hypertension at 6 weeks. Treprostinil was used with rapid clinical improvement. Repeat cardiac catheterization showed dramatic improvement. Both infants were weaned off the drug, representing the first reports of successful short-term treprostinil use in neonates with congenital diaphragmatic hernia.