Abstract
Despite rapid diagnosis, modern surgical technique and intensive pre- and post-operative care, survival of infants with CDH who present within the first 24 hours of life is poor. Some authors suggest that the degree of lung hypoplasia is often the determinant in survival. In our experience almost all infants with CDH can be adequately ventilated following surgery, yet many of these infants subsequently die. After surgery for CDH, 4 infants had blood sampled from pre-and postductal sites. All infants demonstrated R→L shunting suggesting PH. A 5th infant who became progressively hypoxic following surgery underwent cardiac catheterization. No congenital cardiac lesions were found. However, PH with R→L shunting through the foramen ovale and ductus arteriosus (DA) were demonstrated. In addition left pulmonary venous desaturation was present. Both tolazoline (TZL) and chlorpromazine (CPZ) were injected directly into the main pulmonary artery (MPA). Pulmonary artery pressure (PAP) dropped, systemic arterial saturation rose, and pulmonary circulation improved. It was unclear whether the improvement was fortuitous. Subsequently PAP rose and repeat doses of TZL and CPZ administered into the MPA caused little response, 14 hours later the infant died. PH complicating CDH probably occurs frequently. Establish ment of pulmonary normotension may be necessary for survival.
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Pomerance, J., Richardson, C., Collins, P. et al. PULMONARY HYPERTENSION (PH) AND CONGENITAL DIAPHRAGMATIC HERNIA (CDH). Pediatr Res 8, 469 (1974). https://doi.org/10.1203/00006450-197404000-00772
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DOI: https://doi.org/10.1203/00006450-197404000-00772