Pulmonary vascular remodeling.
The maladaptive response of the pulmonary vasculature that occurs in patients with congenital diaphragmatic hernia significantly impacts outcome. Muscularized distal pulmonary arterioles inhibit the ability of the neonate to adjust to extrauterine circulation, resulting in severe pulmonary hypertension. This review summarizes the current state of knowledge regarding normal and abnormal development of the lung vascular system and identifies current and potential therapies directed toward preserving or restoring proper pulmonary vascular function.
Miniati
Doug
D
Division of Pediatric Surgery, Department of Surgery, University of California, San Francisco School of Medicine, San Francisco, California, USA. miniatid@surgery.ucsf.edu
D001160:Arterioles / Q000254:growth & development; D065630:Hernias, Diaphragmatic, Congenital; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000628:therapy; D008168:Lung / Q000098:blood supply*; D011652:Pulmonary Circulation / Q000502:physiology