Pulmonary hypertension and cor pulmonale in cystic fibrosis of the pancreas

Roberta M. Goldring, Alfred P. Fishman, Gerard M. Turino, Herbert I. Cohen, Carolyn R. Denning, Dorothy H. Andersen

Research output: Contribution to journalArticlepeer-review

76 Scopus citations

Abstract

The present study was concerned with the pathogenesis of pulmonary arterialhypertension in 21 patients with the pulmonary complications of cystic fibrosis. Respiratory function tests failed to distinguish between patients with normal pulmonary arterial pressure and those with pulmonary hypertension. On the other hand, the pulmonary arterial pressure was directly related to the degree of hypoxia. Relief of hypoxia, either acutely by breathing high-oxygen mixtures, or chronically by the treatment of respiratory infection and bronchial drainage, returned pulmonary arterial pressure toward, or to, normal. These physiologic observations imply a predominant role for hypoxia in the pathogenesis of pulmonary hypertension in cystic fibrosis, and indicate its potential reversibility in this disease.

Original languageEnglish
Pages (from-to)501-524
Number of pages24
JournalJournal of Pediatrics
Volume65
Issue number4
DOIs
StatePublished - Oct 1964
Externally publishedYes

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