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Published on: January 27, 2023
Cor triatriatum dexter: A rare cause of cyanosis during neonatal period
1Department of Cardiac Science, College of Medicine, King Saud University, Riyadh, Kingdom of Saudi Arabia.
Insights
Cor-triatriatum dexter, a rare congenital heart defect, partitions the right atrium. Surgical resection of the dividing membrane successfully treated a neonate with cyanosis.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Congenital Heart Disease
Background:
- Cor-triatriatum dexter is an extremely rare congenital heart defect.
- It involves the persistence of the right valve of the embryonic sinus venosus, partitioning the right atrium.
- This partitioning creates distinct smooth and trabeculated portions within the atrium.
Observation:
- A 1-week-old infant presented with intermittent central cyanosis.
- Diagnosis was confirmed via echocardiography and bubble contrast study.
- The defect was isolated, with no other cardiac abnormalities.
Findings:
- Initial treatment with cardiac catheterization failed to disrupt the membrane.
- Successful treatment was achieved through open-heart surgery to resect the cor-triatriatum dexter membrane.
- The surgical outcome was uncomplicated.
Implications:
- This case highlights the challenges in managing cor-triatriatum dexter.
- Surgical intervention remains a definitive treatment option for this rare condition.
- Early diagnosis and appropriate management are crucial for improving outcomes in affected neonates.
Abstract:
Cor-triatriatum dexter is an extremely rare congenital heart defect in which there is complete persistence of the right valve of embryonic sinus venosus that results in partitioning of the right atrium into a smooth and trabeculated portion. The smooth portion receives venous blood from inferior vena cava, superior vena cava, and coronary sinus while the trabeculated portion contains the right atrial appendage and the opening of tricuspid valve. We report a 1-week-old child who presented with intermittent episodes of central cyanosis. Echocardiography, established, and bubble contrast study confirmed the diagnosis of an isolated cor-triatriatum dexter. The baby initially underwent an intervention by cardiac catheterization, which was unsuccessful in disrupting the membrane and re-direct the systemic venous flow to the right heart chambers. She subsequently had the cor-triatriatum dexter membrane resected via an uncomplicated open-heart surgery.
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