Dynamics of pulmonary hypertension severity in the first 48 h in neonates with prenatally diagnosed congenital diaphragmatic hernia

Abstract

IntroductionPulmonary hypertension (PH) is one of the major contributing factors to the high morbidity and mortality in neonates with congenital diaphragmatic hernia (CDH). The severity and duration of postnatal PH are an established risk factor for patient outcome; however, the early postnatal dynamics of PH have not been investigated. This study aims to describe the early course of PH in CDH infants, and its relation to established prognostic markers and outcome measures.MethodsWe performed a monocentric retrospective review of neonates with prenatally diagnosed CDH, who received three standardized echocardiographic examinations at 2–6 h, 24, and 48 h of life. The degree of PH was graded as one of three categories: mild/no, moderate, or severe PH. The characteristics of the three groups and their course of PH over 48 h were compared using univariate and correlational analyses.ResultsOf 165 eligible CDH cases, initial PH classification was mild/no in 28%, moderate in 35%, and severe PH in 37%. The course of PH varied markedly based on the initial staging. No patient with initial no/mild PH developed severe PH, required extracorporeal membrane oxygenation (ECMO)-therapy, or died. Of cases with initial severe PH, 63% had persistent PH at 48 h, 69% required ECMO, and 54% died. Risk factors for any PH included younger gestational age, intrathoracic liver herniation, prenatal fetoscopic endoluminal tracheal occlusion (FETO)-intervention, lower lung to head ratio (LHR), and total fetal lung volume (TFLV). Patients with moderate and severe PH showed similar characteristics, except liver position at 24- (p = 0.042) and 48 h (p = 0.001), mortality (p = 0.001), and ECMO-rate (p = 0.035).DiscussionTo our knowledge, this is the first study to systematically assess the dynamics of PH in the first postnatal 48 h at three defined time points. CDH infants with initial moderate and severe PH have a high variation in postnatal PH severity over the first 48 h of life. Patients with mild/no PH have less change in PH severity, and an excellent prognosis. Patients with severe PH at any point have a significantly higher risk for ECMO and mortality. Assessing PH within 2–6 h should be a primary goal in the care for CDH neonates.

Patent ductus arteriosus shunting direction and diameter predict inpatient outcomes in newborns with congenital diaphragmatic hernia

“…Approximately 85% of CDH newborns manifest pulmonary hypertension within the first 48 h of life, with limited treatment options currently available (23,24). Additionally, Leyens et al reported the dynamics of PH in the first 48 h of life at three defined times point of echocardiography and observed a fluctuation in PH severity in approximately two third of all patients (25). We had similar results with those reports, while we not only examined every patient and had a primary diagnosis for the first several hours after birth, but measured changes of PH after medical treatment on the second day or before the surgery within 24 h. On the second echo, the PDA directionality of shunting and diameter changed, which predicted outcomes in this patient population from our results.…”

Spontaneous breathing in selected neonates with very mild congenital diaphragmatic hernia

“…The severity of PH was graded as mild PH (pulmonary arterial pressure [PAP] < ⅔ systemic systolic pressure), moderate PH (⅔ of systemic pressure to systemic pressure), or severe PH (suprasystemic pressure) as previously described 15 . This assessment includes: (a) ductus arteriosus flow pattern, (b) position of the intraventricular septum, and (c) velocity of the tricuspid regurgitation jet if present.…”