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Double outlet right ventricle and left-sided aorta. A previously undescribed association with cor triatriatum and
Insights
This study details a rare congenital heart defect: double-outlet right ventricle with unusual chamber anatomy. The findings highlight a previously undescribed combination of cardiac anomalies in a single patient.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Pediatric Cardiology
Background:
- Double-outlet right ventricle (DORV) is a complex congenital heart defect.
- Viscero-atrial situs solitus with a right-sided right ventricle and left-sided aorta is an uncommon presentation of DORV.
Observation:
- A case of DORV with situs solitus presented with a unique combination of anomalies.
- This included dextrocardia, a subaortic ventricular septal defect, and an ostium secundum atrial septal defect.
Findings:
- The patient exhibited a previously undescribed condition: cor triatriatum with a double right ventricular chamber.
- Absence of pulmonary and aortic stenosis was noted at all levels.
Implications:
- This case expands the spectrum of known DORV presentations.
- It underscores the importance of comprehensive cardiac evaluation in complex congenital heart disease.
- Further research may elucidate the embryological basis for this unique combination of defects.
Abstract:
Double-outlet right ventricle with viscero-atrial situs solitus, right-sided right ventricle and left-sided aorta is an unusual finding. In the present case it was found in association with a cor triatriatum and a double right ventricular chamber, a condition which has not been described previously. Dextrocardia, subaortic ventricular septal defect, atrial septal defect ostium secundum type and absence of pulmonary and aortic stenosis at any level were also noted.