Semin Respir Crit Care Med 1998; 19(3): 259-269
DOI: 10.1055/s-2007-1009403
Copyright © 1998 by Thieme Medical Publishers, Inc.

Congenital and Idiopathic Lung Diseases in Pregnancy

Robert M. Kotloff
  • Program for Advanced Lung Disease and Lung Transplantation, University of Pennsylvania Medical Center, Philadelphia, Pennsylvania
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Publication Date:
20 March 2008 (online)


Pregnancy is associated with a number of physiologic and hormonal changes that are readily handled by the otherwise normal female but may adversely impact on the health of a woman with underlying respiratory disorders. This article explores the relationship between pregnancy and four respiratory disorders of an idiopathic or congenital nature with a propensity to involve women of child-bearing years-cystic fibrosis (CF), lymphangioleiomyomatosis (LAM), sarcoidosis, and primary pulmonary hypertension (PPH). It is apparent that pregnancy poses an unacceptable risk only for women with PPH, for whom maternal mortality exceeds 50%. In contrast, pregnancy is well tolerated by many women with CF of a mild to moderate degree. In more advanced cases of CF, maternal risk appears to be dictated by the degree of cardiopulmonary impairment and not independently heightened by pregnancy. Finally, the hormonal changes of pregnancy may exacerbate disease manifestations in patients with LAM, while possibly having an ameliorating effect on the course of sarcoidosis.