CONGENITAL AND IDIOPATHIC LUNG-DISEASES IN PREGNANCY

Pregnancy is associated with a number of physiologic and hormonal chan ges that are readily handled by the otherwise normal female but may ad versely impact on the health of a woman with underlying respiratory di sorders. 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 pulmonar y hypertension (PPH), It is apparent that pregnancy poses an unaccepta ble 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 ris k appears to be dictated by the degree of cardiopulmonary impairment a nd not independently heightened by pregnancy. Finally, the hormonal ch anges of pregnancy may exacerbate disease manifestations in patients w ith LAM, while possibly having an ameliorating effect on the course of sarcoidosis.