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Hypoplastic left heart syndrome associated with congenital right-sided diaphragmatic hernia and omphalocele
M Nishimura1, A Taniguchi, H Imanaka
1Department of Anesthesiology, Osaka Medical Center, Japan.
Insights
Congenital diaphragmatic hernia (CDH) rarely occurs with hypoplastic left heart syndrome (HLHS). This case report details a newborn with CDH, HLHS, and omphalocele, highlighting the rarity of this combination.
Area of Science:
- Pediatric Surgery
- Neonatal Cardiology
- Medical Genetics
Background:
- Congenital diaphragmatic hernia (CDH) is a serious birth defect impacting lung development.
- Cardiac anomalies are frequently observed in CDH patients, but specific associations vary.
- Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect characterized by underdevelopment of the left heart structures.
Observation:
- A rare case of a female newborn presenting with the triad of CDH, HLHS, and omphalocele was identified.
- The patient underwent surgical repair for omphalocele and the diaphragmatic defect.
- Postoperatively, the patient developed cardiac failure necessitating intervention for HLHS.
Findings:
- The surgical repair of omphalocele and CDH was technically successful.
- Despite surgical intervention for CDH and omphalocele, the patient succumbed to cardiac failure following Norwood's procedure for HLHS.
- This case represents the first documented instance of the simultaneous occurrence of CDH, HLHS, and omphalocele.
Implications:
- This case underscores the complex interplay between major congenital anomalies and their impact on neonatal outcomes.
- The extreme rarity of this combination necessitates further investigation into potential shared etiologies or developmental pathways.
- Understanding such complex presentations is crucial for refining diagnostic and therapeutic strategies in neonatal critical care.
Abstract:
Congenital diaphragmatic hernia (CDH) is associated with a variety of cardiac anomalies. However, its association with hypoplastic left heart syndrome (HLHS) is rare. We treated a female newborn with CDH, HLHS, and omphalocele. The operation for omphalocele and the diaphragmatic defect was successful, although the patient died of cardiac failure after Norwood's operation for HLHS. To our knowledge, this is the first reported case with a combination of these three major anomalies: CDH, HLHS, and omphalocele.