A proposal of new therapeutic strategy for antenatally diagnosed congenital diaphragmatic hernia
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Published on: October 31, 2012
Noboru Inamura1, Akio Kubota, Tohru Nakajima
1Department of Pediatric Cardiology, Osaka Medical Center and Research Institute for Maternal and Child Health, Osaka 594-1101, Japan. inamura@mch.pref.osaka.jp
Insights
Congenital diaphragmatic hernia (CDH) management improved with a new strategy focusing on cardiac function. Maintaining ductus arteriosus patency with nitric oxide (NO) and prostaglandin E1 (PGE1) is crucial for excellent outcomes in severe pulmonary hypertension.
Area of Science:
- Pediatric Surgery
- Neonatal Cardiology
- Critical Care Medicine
Background:
- Congenital diaphragmatic hernia (CDH) presents a significant challenge with historically poor prognoses despite therapeutic advancements.
- Understanding the impact of cardiac function is essential for optimizing treatment strategies in antenatally diagnosed CDH (ADCDH).
Purpose of the Study:
- To evaluate a novel therapeutic strategy for ADCDH, focusing on cardiologic function and circulatory management.
- To assess the role of prostaglandin E1 (PGE1) in managing pulmonary hypertension in ADCDH patients.
Main Methods:
- Cardiac function was assessed in 19 ADCDH cases, divided into groups based on the need for PGE1 to manage pulmonary hypertension.
- Left ventricular end-diastolic dimension index (LVDI) and total pulmonary artery index (TPAI) were measured at birth (day 0) and postnatal day 2.
- The use of inhaled nitric oxide (NO) and PGE1 was recorded.
Main Results:
- The study observed a low mortality rate (1/19) due to cardiac or respiratory failure.
- Patients requiring PGE1 showed smaller LVDI and TPAI on day 0 compared to those not requiring PGE1 and controls.
- The right ventricle compensated for low left ventricular output by maintaining ductus arteriosus patency with NO and PGE1, with LVDI increasing from day 0 to day 2.
Conclusions:
- Maintaining ductus arteriosus patency using NO and PGE1 is critical for achieving excellent clinical outcomes in ADCDH with severe pulmonary hypertension.
- A new therapeutic strategy for ADCDH based on circulatory management, particularly focusing on ductal patency, is proposed.
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.
