The lungs in congenital diaphragmatic hernia: Do we understand?

The long-term follow-up of patients with a congenital diaphragmatic hernia: a broad spectrum of morbidity

“…Apart from the pulmonary vascular abnormalities observed in CDH patients, an altered expression of several factors may be involved in regulation of the vascular tone [84,85]. All these factors together may predispose infants born with CDH for pulmonary hypertension [45,76,77].…”

Cardiopulmonary anatomy and function in long-term survivors of mild to moderate congenital diaphragmatic hernia

“…1,2 However, long-term cardiopulmonary sequelae of CDH survivors are still unclear, and understanding of developmental processes of hypoplasic lung is limited. 3 In the last decades, several studies reported the presence of persisting pulmonary abnormalities in a minority of CDH survivors, 4-8 but recent improvement in treatment led to survival of more severely affected infants, and little is known about these patients. 2 The impact of these anatomic defects on cardiopulmonary function remains unclear: cardiac function is considered normal in isolated CDH survivors, 9 but conflicting results in pulmonary function tests have been reported.…”

Pulmonary vascular morphology in a fetal rabbit model for congenital diaphragmatic hernia

“…It affects 1 in 2,400 births when intrauterine fetal deaths and stillborns are included. 1,3 It is widely believed that herniating viscera act as other space-occupying lesions and impair normal development of the fetal lungs, leading to more or less severe pulmonary hypoplasia. 4 The clinical course of an infant with CDH depends on the degree of pulmonary hypoplasia as well as the degree of pulmonary hypertension, in turn, related to changes in pulmonary vascular tree.…”