Pulmonary-esophageal variceal bleeding: a unique presentation of partial cor triatriatum sinistrum
Fortune O Alabi1, Manuel Hernandez2, Francis G Christian1
1Department of Critical Care Medicine, Florida Hospital Celebration Health, Celebration, FL 34747, USA.
Insights
Cor triatriatum sinistrum, a rare congenital heart defect, involves a membrane dividing the left atrium. This case highlights its association with variceal bleeding and pulmonary venous obstruction.
Area of Science:
- Cardiology
- Congenital Heart Disease
- Medical Imaging
Background:
- Cor triatriatum sinistrum is a rare congenital anomaly characterized by a dividing membrane within the left atrium.
- This membrane can lead to pulmonary venous obstruction and associated complications.
- It is crucial to understand its varied presentations and diagnostic challenges.
Purpose of the Study:
- To report a unique case of cor triatriatum sinistrum presenting as recurrent hematemesis.
- To illustrate the diagnostic utility of CT imaging in identifying this rare condition.
- To discuss the implications of pulmonary venous obstruction and collateralization in this context.
Main Methods:
- Case report of a young adult male with recurrent hematemesis.
- Computed Tomography (CT) imaging to evaluate cardiac and vascular anatomy.
- Review of relevant medical literature on cor triatriatum sinistrum and variceal bleeding.
Main Results:
- CT imaging revealed cor triatriatum sinistrum with a vertical membrane causing complete obstruction of right pulmonary venous drainage.
- The patient presented with recurrent hematemesis secondary to variceal bleeding.
- Extensive pulmonary-systemic arterial collateralization was observed, suggesting reversed flow in the right pulmonary artery.
Conclusions:
- Cor triatriatum sinistrum can present with atypical symptoms such as hematemesis due to secondary complications like variceal bleeding.
- CT imaging is vital for diagnosing this rare congenital heart defect and associated vascular abnormalities.
- Understanding the hemodynamic consequences, including collateral formation, is essential for patient management.
Abstract:
Cor triatriatum sinistrum is a rare congenital disorder defined as a division of the left atrium by a diaphragmatic membrane resulting in two left atrial chambers. The membranous division of the atrium can be partial or complete and can affect either atrium, with involvement of the right atrium referred to as cor triatriatum dexter. The presence of fenestrations within the membrane allows for communication and forward passage of blood into the true atrium. Absence of fenestrations leads to early symptomatic engorgement of the lungs. We report the case of a young adult male presenting with recurrent hematemesis due to variceal bleeding. On CT imaging the patient was found to have cor triatriatum sinistrum, with a vertical membrane resulting in total obstruction of the pulmonary venous drainage on the right, with normal pulmonary venous drainage on the left. There was extensive pulmonary-systemic arterial collateralization to the right lung suggesting retrograde filling of the right pulmonary artery with effective flow reversal in the right lung.
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