https://orcid.org/0000-0003-3274-9561
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Guoliang Yang, Yun Zhang, Zheng Liu, The role of natural compensatory mechanisms in complex congenital heart disease: haemodynamic evolution and clinical outcomes in a case of Cor triatriatum sinistrum syndrome, European Heart Journal, Volume 45, Issue 35, 14 September 2024, Page 3297, https://doi.org/10.1093/eurheartj/ehae354
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A 19-year-old woman with a history of exertional dyspnoea and cyanosis since childhood was diagnosed with congenital heart disease, specifically Cor triatriatum sinistrum (CTS) syndrome. Cor triatriatum sinistrum is a rare cardiac anomaly where a fibrous membrane divides the left atrium into two chambers.1 Despite experiencing severe hypoxia symptoms in infancy, she grew normally into adulthood, with pulmonary arterial hypertension being the only lasting effect. Her initial assessment showed good development, with a resting oxygen saturation of 99%. However, the electrocardiogram revealed the presence of right bundle branch block (Panel A). Transthoracic echocardiography showed right heart enlargement, pulmonary artery dilation, and evidence of CTS with outflow obstruction (Panels B and C; Supplementary data online, Videos S1 and S2). Transoesophageal echocardiography and CT angiography confirmed multi-hole atrial septal defects, with a persistent left superior vena cava (PLSVC) anomalously connecting to the left brachiocephalic vein and left upper pulmonary vein (Panels D and E; Supplementary data online, Videos S3 and S4). Haemodynamically, the left upper pulmonary vein drained into the PLSVC, which then connected to the innominate vein. Because the accessory chambers belong to high-pressure cavities, oxygenated blood enters the right atrium during atrial emptying through the atrial septal defect and the PLSVC. Secondary pulmonary hypertension caused right-to-left shunting during atrial filling, especially when the largest atrial septal defect opening was located between the true atrium and right atrium, which unexpectedly alleviated the patient’s hypoxia symptoms. This haemodynamic adaptation explains her normal development and reduced cyanotic symptoms in adulthood. The patient underwent surgical correction for triatriatum syndrome (Panel F), which involved ligating the PLSVC, removing the intracardiac membrane, and closing the atrial septal defect with a patch. After surgery, her haemodynamics normalized, the intracardiac shunt resolved, and exertional dyspnoea symptoms disappeared. She recovered and was discharged. This case highlights the interaction between congenital anomalies and physiological adaptations. Detailed imaging diagnosis and haemodynamic evaluation contribute to a better understanding of the pathophysiological mechanisms of the disease and help tailor personalized treatment strategies for patients.
