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The lung following repair of congenital diaphragmatic hernia

, : The lung following repair of congenital diaphragmatic hernia. Journal of Pediatrics 90(3): 405-414

To determine the effects of the pulmonary hypoplasia present at birth in infants with congenital diaphragmatic hernia upon subsequent development of the lung, 19 patients who had undergone surgical repair before the age of 1 yr were studied at ages 6-18 yr. Total lung capacity and vital capacity averaged 99% of predicted value. Diffusing capacity for CO was normal. Forced expiratory volume in 1 s averaged 89% of predicted value and 80% of vital capacity. Total respiratory system conductance and maximum expiratory flow volume curves obtained during air and He-O2 breathing were normal. 133Xe radiospirometry performed in 9 patients revealed equal distribution of lung volumes on the 2 sides. Ventilation to the hernia side was reduced in only 2 patients. Blood flow to the hernia side was reduced in all 9 patients. Chest radiographs supported the physiologic observations. These findings are consistent with the persistence of a reduction in the number of branches or generations of pulmonary arteries and bronchi on the side of the hernia. Since a substantial part of the vascular resistance resides in peripheral vessels, this developmental abnormality influences the distribution of pulmonary blood flow, although it has little effect on tests reflecting airway resistance or the distribution of ventilation.

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Accession: 006712401

PMID: 839333

DOI: 10.1016/s0022-3476(77)80702-6

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