Key result
Resection of a calcified cor triatriatum sinister membrane resolves severe post-capillary pulmonary hypertension.
Why the study?
Cor triatriatum sinister is an exceptionally rare congenital cardiac anomaly whose restrictive forms typically present in infancy or early childhood, making survival into adolescence with a 2-3 mm fenestration unusual.
Case Report (n=1)
No
Answers are grounded in this paper and cite their sources.
This case demonstrates that severely restrictive cor triatriatum sinister can rarely present in adolescence mimicking refractory asthma, and invasive haemodynamic confirmation of reversible post-capillary pulmonary hypertension justifies successful surgical correction.
Cor triatriatum sinister is an exceptionally rare congenital cardiac anomaly [1]. While restrictive forms are typically present in infancy or early childhood, survival into adolescence with a 2-3 mm fenestration is unusual [2, 3]. We report a 14-year-old male, misdiagnosed with refractory asthma for years, who presented with palpitations. Imaging and catheterisation revealed severe post-capillary pulmonary hypertension with echocardiographic evidence of right-sided chamber dilatation, D-shaped left ventricular configuration, moderate tricuspid regurgitation, and an estimated pulmonary artery pressure of 85 mmHg. Cardiac catheterisation showed PA pressure of 50/30 mmHg (mean 39 mmHg), PCWP of 28 mmHg, Qp/Qs of 1.14, and a favourable PVR/SVR ratio of 0.12, indicating reversibility. Successful surgical resection of a calcified membrane was performed via right anterolateral thoracotomy. This case underscores the importance of considering cor triatriatum sinister in adolescents with unexplained respiratory symptoms or wheezing and highlights the value of correlating echocardiographic evidence of obstruction with invasive haemodynamic assessment before surgery [4, 5].
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Acar et al. (2026) conducted a case report in Restrictive cor triatriatum sinister with severe post-capillary pulmonary hypertension mimicking refractory asthma (n=1). Surgical resection of calcified left atrial membrane via right anterolateral thoracotomy, followed by postoperative enalapril and metoprolol was evaluated on Resolution of left atrial obstruction and reduction in right ventricular pressures. Surgical resection of a calcified cor triatriatum sinister membrane (2-3 mm fenestration) resolved severe post-capillary pulmonary hypertension (PCWP 28 mmHg) in a 14-year-old male.
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