A rare late presentation of restrictive cor triatriatum sinister in a 14-year-old male mimicking refractory asthma

Abstract

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].