Outcomes of Congenital Diaphragmatic Hernia in the Modern Era of Management

Abstract

Objective To identify clinical factors associated with pulmonary hypertension and mortality in patients with congenital diaphragmatic hernia (CDH). Study design A prospective cohort of neonates with a diaphragm defect identified at one of seven collaborating medical centers was studied. Echocardiograms were performed at one month and three months of age and analyzed at a central core by two cardiologists independently. Degree of pulmonary hypertension and survival were tested for association with clinical variables using Fischers exact test, chi-square and regression analysis. Results 220 patients met inclusion criteria. Worse pulmonary hypertension measured at one month of life was associated with higher mortality. Other factors associated with mortality were need for extracorporeal membrane oxygenation (ECMO), patients inborn at the treating center and patients with a prenatal diagnosis of CDH. Interestingly, patients with right sided CDH did not have worse outcomes. Conclusions Severity of pulmonary hypertension is associated with mortality in CDH. Other factors associated with mortality were birth weight, gestational age at birth, inborn status and need for ECMO.

Effect of corticosteroids and lung ventilation in the VEGF and NO pathways in congenital diaphragmatic hernia in rats

“…Ideal treatment for these cases still remains to be discussed and investigated [18,19]. Most of the newborns with CDH need mechanical ventilation and it can lead to inflammation and injury at short and long term.…”

PBX transcription factors drive pulmonary vascular adaptation to birth

“…Congenital diaphragmatic hernia (CDH) is a common congenital anomaly that occurs in 1 in 3,500 to 5,000 live births (1,2). Despite improvements in the care of infants with CDH, the mortality rate associated with the disease continues to be 20% in isolated cases and 50% in complex cases (3)(4)(5). The primary cause of the high mortality rate is a lethal combination of pulmonary hypoplasia and pulmonary hypertension (PH) (6,7).…”

STATs in Lung Development: Distinct Early and Late Expression, Growth Modulation and Signaling Dysregulation in Congenital Diaphragmatic Hernia

“…Concomitantly with herniation, severe pulmonary hypoplasia and pulmonary hypertension (PH) develop [4,5]. In fact, these have proven to be the most complex aspects to therapeutically address in CDH [6,7]. The nitrofen animal model has contributed extensively to CDH research.…”