出生前诊断的先天性膈疝的新治疗方案

作     者：Inamura N. Kubota A. Nakajima T. 郭战宏 

作者机构：Department of Pediatric C ardiology Osaka Medical Center Research Institute for Maternal and Child Healt h Osaka 594-1101 Japan 

出 版 物：《世界核心医学期刊文摘（儿科学分册）》 (Dkgest of the World Latest Medical Information)

年 卷 期：2006年第2卷第1期

页      面：43-43页

学科分类：1002[医学-临床医学] 100202[医学-儿科学] 10[医学] 

主　　题：出生前诊断 先天性膈疝 肺动脉指数 肺动脉高压 舒张末期内径 心脏功能 动脉直径 舒张期 前列腺素 

摘      要：Purpose: The prognosis of antenatally diagnosed congenital diaphragmatic herni a (ADCDH) is still very poor despite of innovation of various therapeutics. The authors reviewed their new therapeutic strategy of ADCDH from a viewpoint of car diologic function. Methods: The cardiac function in 19 cases of ADCDH was review ed. 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 pu lmonary hypertension. The left ventricular (LV) end-diastolic dimension (LV dia stolic diameter index [LVDI]) and bilateral pulmonary arterial diameters (total pulmonary artery index [TPAI]) were measured on days 0 and 2. Result: Only 1 pat ient died of cardiac or respiratory failure, and the survivors’postoperative co urse 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 (+) wer e significantly smaller than those in PG (-) and the controls. The LVDI increas ed from postnatal day 0 to day 2 in both PG (+) and PG (-). Although the LV wa s too small to output enough volume, the right ventricle successfully compensate d for the low output through the ductus arteriosus, kept patent by NO and PGE1. Conclusion: For ADCDH with sever pulmonary hypertension, keeping patent ductus a rteriosus 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.