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Cor biloculare and associated malformations
Insights
This study details four rare cases of cor biloculare, highlighting similar associated anomalies like severe heart malformations and visceral heterotaxia. Findings suggest altered embryonic blood flow may cause this complex congenital heart defect.
Area of Science:
- Cardiology
- Developmental Biology
- Clinical Pathology
Background:
- Cor biloculare is a rare congenital heart anomaly characterized by a two-chambered heart.
- Previous reports show diverse associated anomalies, making consistent etiological links challenging.
Observation:
- Four unusual cases of cor biloculare were analyzed over a four-year period.
- All cases presented with similar anomalies: severe pulmonic valve malformations, pulmonary artery atresia, and visceral heterotaxia.
- Uncomplicated pregnancies and normal gestational histories were noted in all cases.
Findings:
- A high incidence of anomalous venous return (pulmonary and systemic) and visceral heterotaxia was observed.
- Three cases exhibited anomalous pulmonary venous drainage, while one had anomalous systemic venous drainage.
- All cases involved polysplenia or asplenia, indicating complex developmental disruptions.
Implications:
- The consistent constellation of anomalies suggests a specific developmental pathway for cor biloculare.
- Altered embryonic blood flow is proposed as a key etiological factor in the development of these complex malformations.
- Further research into early embryonic hemodynamics may elucidate the pathogenesis of cor biloculare and associated defects.
Abstract:
We report the clinical and autopsy findings of four unusual cases of the rate entity, cor biloculare, collected within a 4-year period. The cases are interesting for (1) the striking similarity of the associated anomalies, in contrast within the diversity of associated anomalies reported before; (2) the greater incidence of anomalous pulmonary and systemic venous return, pulmonic valve malformations and visceral heterotaxia than reported before; and (3) the normal, full-term, uncomplicated pregnancies. Family and gestational histories were unrevealing. All four cases had severe pulmonic valve malformations, pulmonary artery atresia, and visceral heterotaxia. Three of the four had anomalous pulmonary venous drainage and the fourth had anomalous systemic venous drainage. All four cases had polysplenia or asplenia. Correlating these associated anomalies with stages of embryonic development and with various studies of altered embryonic blood flow, suggests an etiologic role for altered blood flow in the development of this complex malformation.