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Advancing cor triatriatum in Fontan circulation
Yusuke Misumi1, Takaya Hoashi, Koji Kagisaki
1Department of Pediatric Cardiovascular Surgery, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan.
The Annals of Thoracic Surgery
|March 26, 2013
Summary
Cor triatriatum, a rare congenital heart defect, was diagnosed in a 14-year-old female ten years after a Fontan procedure. Surgical resection of the abnormal left atrial septum successfully resolved the condition.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- A 14-year-old female with complex congenital heart disease, including mitral atresia and double outlet right ventricle, previously underwent a staged Fontan completion.
- The patient presented with symptoms suggestive of a previously undiagnosed cardiac anomaly years after initial surgical palliation.
Observation:
- Transthoracic echocardiography revealed abnormal blood flow acceleration within the left atrium.
- Cardiac catheterization confirmed a significant pressure gradient between the common pulmonary venous chamber and the left atrium, indicative of cor triatriatum.
Findings:
- Cor triatriatum, characterized by an abnormal septum dividing the left atrium, was diagnosed in a patient with a history of complex congenital heart disease and Fontan palliation.
- Surgical intervention involved the resection of the anomalous intra-atrial septum via a left-side right atriotomy.
Implications:
- Successful surgical correction of cor triatriatum in this complex pediatric cardiac patient highlights the importance of timely diagnosis and intervention.
- This case underscores the potential for late-onset or unmasked intracardiac anomalies in patients with complex congenital heart disease following Fontan procedures.
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