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Pulmonary vascular pathology in congenital diaphragmatic hernia

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Abstract

Initial pathologic examination of the lungs in infants who died with congenital diaphragmatic hernia (CDH) reveal a decrease in size and weight, with the ipsilateral lung being smaller and showing distorted distribution of segmental airways. These pathologic findings have led to an assumption that the high mortality seen following repair of a diaphragmatic hernia was due to pulmonary hypoplasia. In recent years, the abundant pathologic and physiologic data on lungs in CDH clearly emphasize the role played by the pulmonary vasculature in the survival of CDH. An exaggerated vasoconstrictive response of an abnormally hypertrophied pulmonary vascular bed leading to increased pulmonary vascular resistance appears to be the most important mechanism leading to the often fatal hypoxemia seen in these neonates.

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Authors and Affiliations

  1. Department of Pediatric Surgery, Stanford University Medical Center, 94305, Stanford, CA, USA

    Stephen J. Shochat

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  1. Stephen J. Shochat
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Shochat, S.J. Pulmonary vascular pathology in congenital diaphragmatic hernia. Pediatr Surg Int 2, 331–335 (1987). https://doi.org/10.1007/BF00175644

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  • DOI: https://doi.org/10.1007/BF00175644

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