ZainEdeen Zyadah, Joyce Morcos, Alaa Alresheq, Salem K. Qupp, Ahmad Fatayer, Jahed Bushnaq
Al-Quds University. Palestine Medical Complex. Washington University of Health and Science.
Palestine and Belize
Frontiers in Cardiovascular Medicine
Front Cardiovasc Med 2026; 13:
DOI: 10.3389/fcvm.2026.1785372
Abstract
Background: Cor Triatriatum sinister (CTS) is a rare congenital cardiac anomaly caused by a fibromuscular membrane subdividing the left atrium, potentially leading to impaired left ventricular inflow, pulmonary venous hypertension, and heart failure. Clinical presentation is highly variable and may mimic primary respiratory disease, resulting in delayed diagnosis, particularly in older children and adolescents.
Case summary: A 13-year-old previously healthy boy presented with acute hypoxic respiratory failure following one week of progressive exertional dyspnea. Initial findings of elevated jugular venous pressure and bilateral pulmonary congestion with low inflammatory markers suggested a cardiogenic etiology. Bedside lung ultrasonography demonstrated diffuse pulmonary oedema, and transthoracic echocardiography revealed a severely obstructive supramitral membrane consistent with cor Triatriatum sinister and secondary pulmonary hypertension. After stabilization with noninvasive ventilation and diuresis, the patient underwent minimally invasive surgical excision of the accessory membrane, resulting in complete relief of obstruction. Postoperative recovery was uneventful, with sustained clinical and echocardiographic improvement on follow-up.
Discussion: This case illustrates the diagnostic challenge posed by CTS when presenting outside infancy and highlights the importance of early cardiac evaluation in pediatric patients with atypical respiratory presentations. Prompt use of bedside ultrasound and echocardiography enabled timely diagnosis and definitive management, leading to an excellent outcome.
Conclusion: CTS, although rare, should be considered in children and adolescents presenting with unexplained pulmonary oedema or hypoxemia. Early recognition and surgical correction are associated with excellent short- and long-term outcomes.
Category
Class II. Pulmonary Hypertension Associated with Pulmonary Vein Stenosis
Symptoms and Findings Associated with Pulmonary Vascular Disease
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes