Abstract
In a prospective study of 58 infants with CDH, there were 30 survivors who demonstrated ductal shunting, reversible with hyperventilation and 28 non survivors with severe hypercarbia and hypoxemia which could not be reversed with either vasodilators or hyperventilation (mean airway pressure >20cms H2O, resp. rates >40/min). Detailed morphcrnetric analysis of the post mortem lung was carried out in 7 infants. Pulmonary vascular changes were mild relative to infants with persistent pulmonary hypertension (PPH) (J Pediatr 98:962,1981*) but comparable to a previously reported case of CDH. (Brit J Surg 58:362, 1971). Considerably more severe pulmonary hypoplasia was identified in 3 patients in whom lung volumes were available and total alveolar number could be calculated. (Newborn controls Am Rev Resp Pis 129:208,1984)**
Thus, while there is increased muscularity in the pulmonary vascular bed of these severely affected infants, the irreversible hypoxemia and hypercarbia seen in life may be better explained by deficiency in number of alveoli and accompanying arteries in both ipsilateral (I) and contralateral (C) lungs.
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Bohn, D., Tamura, M., Perrin, D. et al. 1344 PULMONARY HYPOPLASIA AND VASCULAR ABNORMALITIES IN CONGENITAL DIAPHRAGMATIC HERNIA (CDH). Pediatr Res 19, 334 (1985). https://doi.org/10.1203/00006450-198504000-01368
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DOI: https://doi.org/10.1203/00006450-198504000-01368