PMID: 9445506Jan 31, 1998Paper

Lung volume, pulmonary vasculature, and factors affecting survival in congenital diaphragmatic hernia

Pediatrics
D W Thibeault, B Haney

Abstract

There is a wide variation in published mortality from congenital diaphragmatic hernia (CDH). The prevailing opinion is that this variation is related directly to the degree of pulmonary hypoplasia. Our aim was to test the hypothesis that other factors are important for outcome. The specific objectives of this study were: 1) to quantitate the degree of lung hypoplasia and pulmonary arterial wall thickness in infants eligible for, and treated with, extracorporeal membrane oxygenation (ECMO), using postmortem analysis of lung DNA, wet lung weight, lung volume, and vessel morphometrics; 2) to correlate the degree of lung hypoplasia and vascular changes with functional tests of oxygenation and estimated right ventricular systolic pressures (RVSP); 3) to determine the minimum lung volume necessary for survival; and 4) to determine contributory clinical factors as potential causes of death in ECMO-treated infants with CDH. We retrospectively analyzed all 90 infants with CDH admitted consecutively over a 9-year period to a children's hospital with an ECMO program. Infants were categorized as lived or died, with or without ECMO. Indication for ECMO was an evolving process; however, in general, it was the therapy of last resort for pulmo...Continue Reading

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