A proposal of new therapeutic strategy for antenatally diagnosed congenital diaphragmatic hernia

Purpose

The prognosis of antenatally diagnosed congenital diaphragmatic hernia (ADCDH) is still very poor despite of innovation of various therapeutics. The authors reviewed their new therapeutic strategy of ADCDH from a viewpoint of cardiologic function.

Methods

The cardiac function in 19 cases of ADCDH was reviewed. The patients, at the age of 0 days, were divided into 2 groups, PG (+) and PG (−), according to the requirement of prostaglandin E₁ (PGE₁) to attenuate pulmonary hypertension. The left ventricular (LV) end-diastolic dimension (LV diastolic diameter index [LVDI]) and bilateral pulmonary arterial diameters (total pulmonary artery index [TPAI]) were measured on days 0 and 2.

Result

Only 1 patient died of cardiac or respiratory failure, and the survivors' postoperative course was uneventful. Eleven patients needed inhalation of nitric oxide (NO), and in 9 of those, PGE₁ was administered. The LVDI and TPAI of day 0 in PG (+) were significantly smaller than those in PG (−) and the controls. The LVDI increased from postnatal day 0 to day 2 in both PG (+) and PG (−). Although the LV was too small to output enough volume, the right ventricle successfully compensated for the low output through the ductus arteriosus, kept patent by NO and PGE₁.

Conclusion

For ADCDH with sever pulmonary hypertension, keeping patent ductus arteriosus with NO and PGE₁ plays a critical role in obtaining excellent clinical outcome. Thus, the authors proposed a new therapeutic strategy for ADCDH based on a circulatory management.