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Cor triatriatum sinistrum: presentation of syncope and atrial tachycardia
Malcolm Avari1, Sunil Nair2, Zofia Kozlowska2
1James Paget University Hospitals NHS Foundation Trust, Great Yarmouth, UK.
Insights
A rare congenital heart defect, cor triatriatum sinistrum (CTS), can cause syncope and tachycardia. Surgical repair is the definitive treatment for this unusual cardiac anomaly.
Area of Science:
- Cardiology
- Congenital Heart Disease
Background:
- Cor triatriatum sinistrum (CTS) is a rare congenital cardiac anomaly characterized by an intra-atrial septum dividing the left atrium.
- Diagnosis has increased due to advancements in imaging, with symptoms varying based on fenestration size.
Observation:
- A 39-year-old male presented with syncope and narrow complex tachycardia.
- Transesophageal echocardiography revealed CTS and an associated atrial septal defect (ASD).
Findings:
- The patient's symptoms were attributed to CTS, a condition that can mimic mitral stenosis.
- Surgical intervention, including CTS repair and ASD closure, was successfully performed.
Implications:
- This case highlights CTS as an unusual cause of syncope and tachycardia.
- Early diagnosis and surgical management are crucial for favorable outcomes in CTS patients.
- CTS can occur in isolation or with other cardiac abnormalities, necessitating comprehensive evaluation.
Abstract:
We present a rather unusual cause for syncope associated with atrial tachycardia. A man aged 39 years presented with an episode of syncope and narrow complex tachycardia. Further investigations, including transoesophageal echocardiography, identified cor triatriatum sinistrum (CTS), a rare congenital abnormality characterised by the atrium being divided by a fibrous membrane. Although it is rare, there has been an increase in diagnosis due to developments in diagnostic imaging techniques. Symptoms are related to the size of fenestrations within the fibrous membrane. Presenting symptoms can mimic those seen in mitral stenosis. It is a condition that can occur in isolation, but it can also be associated with other cardiac abnormalities such as an atrial septal defect (ASD) (as in this case). Surgery is the definitive treatment (this man had surgical repair of CTS and closure of ASD) and should be considered at any age if there are any associated symptoms or complications.
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