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Updated: Apr 9, 2026
08:42
Cox-Maze IV Procedure Concomitant with Valvular Surgery In Situs Inversus Dextrocardia: A Single-Center Experience in China
Published on: February 11, 2022
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Summary
This case report details a rare fetal condition, ectopia cordis, diagnosed at 23 weeks gestation. The study correlates ultrasound and autopsy findings of this complex congenital malformation.
Area of Science:
- Medical Genetics
- Fetal Medicine
- Congenital Malformations
Background:
- Ectopia cordis is a rare congenital malformation where the heart is abnormally positioned outside the thorax.
- It is often associated with Pentalogy of Cantrell, a complex syndrome involving multiple defects.
- The incidence is approximately 1 in 100,000 live births in developed countries.
Observation:
- A case of Pentalogy of Cantrell variant in a 23-week fetus was identified.
- Ultrasound revealed ectopia cordis, intracardiac defects, and an omphalocele containing liver and intestines.
- Autopsy confirmed a cleft sternum, thoracic wall defect, and the ectopic heart (thoracic type).
Findings:
- Detailed cardiac examination showed a double outlet right ventricle and a complete atrioventricular septal defect.
- The omphalocele contained the liver and intestines.
- Ultrasound findings were correlated with autopsy results.
Implications:
- This case highlights the importance of detailed fetal anomaly scans for diagnosing complex congenital conditions.
- Accurate prenatal diagnosis aids in genetic counseling and management decisions.
- Understanding the spectrum of Pentalogy of Cantrell is crucial for clinical practice.
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