Seminars in Pediatric Surgery
Volume 16, Issue 2 , Pages 80-87, May 2007

Pulmonary vascular remodeling

  • Doug Miniati, MD

      Affiliations

    • Corresponding Author InformationAddress reprint requests and correspondence: Doug Miniati, MD, UCSF Division of Pediatric Surgery, 513 Parnassus Avenue, HSW-1601, Box 0570, San Francisco, CA 94143-0570.

Division of Pediatric Surgery, Department of Surgery, University of California, San Francisco School of Medicine, San Francisco, California.

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.

Keywords: Congenital diaphragmatic hernia, Lung, Vascular remodeling, Development, Pulmonary hypertension, Vasculogenesis

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PII: S1055-8586(07)00003-0

doi:10.1053/j.sempedsurg.2007.01.002

Seminars in Pediatric Surgery
Volume 16, Issue 2 , Pages 80-87, May 2007

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