Cor Triatriatum Clinical Management and Pathophysiology

Cor triatriatum is a rare congenital cardiac anomaly characterised by a fibromuscular membrane subdividing an atrium into two distinct chambers. In the more prevalent left-sided form (sinister), the membrane separates pulmonary venous inflow from the true left atrium, whereas right-sided variants (dextrum) bisect the right atrium. Fenestrations within this membrane govern the degree of obstruction and create a pressure gradient that mirrors mitral stenosis physiology, leading to pulmonary venous hypertension, right-heart strain and variable clinical presentations. Neonates with restrictive membranes may develop early cyanosis, while adults often present with exertional dyspnoea, exercise intolerance, atrial fibrillation or cardioembolic stroke. Definitive diagnosis relies on multimodal imaging, notably two- and three-dimensional transthoracic and transoesophageal echocardiography, complemented by cardiac MRI or CT for detailed anatomical mapping. Surgical resection of the membrane remains the cornerstone of treatment in symptomatic or obstructive cases, with selected anatomies amenable to catheter-based balloon dilatation. Anticoagulation management is guided by arrhythmia burden and stroke risk, frequently prompting early intervention in patients with atrial fibrillation. A multidisciplinary strategy integrating congenital cardiac surgeons, electrophysiologists and imaging specialists is essential for optimising long-term outcomes.

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Cor Triatriatum Clinical Management and Pathophysiology publication trend

The graph below shows the total number of articles in cor triatriatum clinical management and pathophysiology across all publications each year (not limited to Nature Index journals).

Technical terms

Cor triatriatum: A congenital condition in which an atrium is divided by a membrane, resulting in three atrial chambers.

Fibromuscular membrane: A connective tissue structure that partitions the atrial chamber and governs intracavitary flow.

Fenestration: An opening within the atrial membrane that permits blood flow and influences the pressure gradient.

Pressure gradient: The difference in pressure across the membrane, which can mimic valvular stenosis physiology.

Transoesophageal echocardiography: High-resolution ultrasound imaging performed via the oesophagus to visualise cardiac anatomy and membrane characteristics.

References

1. A Systematic Review of a Long-forgotten Cause of Atrial Fibrillation and Stroke: Cor Triatriatum. Cureus (2019).
2. Cor Triatriatum Sinister diagnosed in adult life with three dimensional transesophageal echocardiography. BMC Cardiovascular Disorders (2010).
3. Cathether-based interventional strategies for cor triatriatum in the adult – feasibility study through a hybrid approach. BMC Cardiovascular Disorders (2015).
4. Cor Triatriatum Sinister Presenting as Cardioembolic Stroke in a Young Woman. Diagnostics (2022).