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Prognostic factors of congenital diaphragmatic hernia accompanied by cardiovascular malformation
Shigehiro Takahashi1, Haruhiko Sago, Yutaka Kanamori
1Division of Neonatology, Center for Maternal-Fetal and Neonatal Medicine, National Center for Child Health and Development, Tokyo, Japan. takahashi-s@ncchd.go.jp
Insights
Infants with congenital diaphragmatic hernia and cardiovascular malformation have a poor prognosis. Severe cardiovascular malformations significantly increase mortality and morbidity risks compared to the hernia
Area of Science:
- Pediatric Surgery
- Neonatal Cardiology
- Congenital Malformations
Background:
- Congenital diaphragmatic hernia (CDH) frequently co-occurs with cardiovascular malformations (CVM).
- Limited data exists on prognostic factors for infants with concurrent CDH and CVM.
- This study investigates CDH with CVM in infants.
Purpose of the Study:
- To evaluate the prognosis of infants diagnosed with both CDH and CVM.
- To identify factors influencing mortality and morbidity in this patient group.
Main Methods:
- Retrospective cohort study utilizing Japanese CDH patient data (2006-2010).
- Examined frequency and outcomes of CVM in infants with CDH.
- Compared severity of CDH and CVM as predictors of mortality/morbidity.
Main Results:
- CVM was present in 12.3% of 614 CDH infants.
- Severe CVM occurred in 66.1% of affected infants.
- Overall survival was 46.4%; survival without morbidity was 23.2%.
- Severe CVM strongly predicted mortality (aOR 7.69) and morbidity (aOR 7.93) compared to CDH severity.
Conclusions:
- Infants with CDH and CVM face a poor prognosis.
- Cardiovascular malformation severity is a critical predictor of adverse outcomes.
- Early assessment of CVM severity is crucial for managing these complex cases.
Background:
Congenital diaphragmatic hernia is associated with cardiovascular malformation. Many prognostic factors have been identified for isolated congenital diaphragmatic hernia; however, reports of concurrent congenital diaphragmatic hernia and cardiovascular malformation in infants are limited. This study evaluated congenital diaphragmatic hernia associated with cardiovascular malformation in infants. Factors associated with prognosis for patients were also identified.
Methods:
This retrospective cohort study was based on a Japanese survey of congenital diaphragmatic hernia patients between 2006 and 2010. Frequency and outcome of cardiovascular malformation among infants with congenital diaphragmatic hernia were examined. Severity of congenital diaphragmatic hernia and cardiovascular malformation were compared as predictors of mortality and morbidity.
Results:
Cardiovascular malformation was identified in 76 (12.3%) of 614 infants with congenital diaphragmatic hernia. Mild cardiovascular malformation was detected in 19 (33.9%) and severe cardiovascular malformation in 37 (66.1%). Their overall survival rate at discharge was 46.4%, and the survival rate without morbidity was 23.2%. Mortality and morbidity at discharge were more strongly associated with severity of cardiovascular malformation (adjusted OR 7.69, 95%CI 1.96-30.27; adjusted OR 7.93, 95%CI 1.76-35.79, respectively) than with severity of congenital diaphragmatic hernia.
Conclusions:
The prognosis for infants with both congenital diaphragmatic hernia and cardiovascular malformation remains poor. Severity of cardiovascular malformation is a more important predictive factor for mortality and morbidity than severity of congenital diaphragmatic hernia.
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