Cor Triatriatum Sinister With an Intact Interatrial Septum and a Decompressing Vein in a Toddler

Objectives

Cor triatriatum sinister is a very rare cardiac anomaly that may lead to pulmonary hypertension, right ventricular dilation, and eventually right heart failure.

Methods

The authors report a procedure performed for a toddler who was misdiagnosed with asthma as an infant. She had progressive respiratory symptoms and was found to have cor triatriatum sinister with a restrictive communication, decompressing vertical vein, severe tricuspid regurgitation, and severe right ventricular dysfunction.

Results

The surgical repair, shown in the video, was uneventful and the right ventricular function was normalized on follow-up echocardiograms.

Conclusion

To the authors’ knowledge, this is the first report of cor triatriatum sinister with a restrictive fenestration, a decompressing vertical vein, an intact interatrial septum, and severe tricuspid regurgitation in a toddler. The treatment was successful as demonstrated by the follow-up echocardiograms, which show normalization of the ventricular function and pulmonary artery pressure.

This educational content was originally presented during the STSA 64th Annual Meeting. This content is published with the permission of the STSA. For more information on the STSA and its next Annual Meeting, please click here.