Congenital diaphragmatic hernia (CDH) is a rare disease, which affects 1 in 2,500 newborns. Congenital diaphragmatic hernia can interfere with the normal development of the pulmonary parenchyma and vascular bed, and in severe cases, it can lead to the development of severe pulmonary arterial hypertension (PAH) and right ventricular failure. We present a neonate with CDH who developed severe PAH and right ventricular dysfunction and was managed with a unique strategy combining venoarterial extracorporeal membrane oxygenation, prostaglandin E1, and a variety of PAH therapies.
A 33-week gestation, 1.75-kg female infant with mitral stenosis/aortic atresia variant of hypoplastic left heart syndrome and severe ventriculo-coronary connections underwent surgical septectomy and bilateral pulmonary artery banding at five weeks of age (2.10 kg). After separation from bypass, she developed hemodynamic instability requiring venoarterial extracorporeal membrane oxygenation support. She was listed for heart transplantation and transplanted after three days of support with an oversized heart (4.7:1 donor–recipient weight ratio).
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.