Rare Adult Survival with TAPVR, ASD, and Cor Triatriatum Sinister: A Case Report

Abstract

Background: Total anomalous pulmonary venous return (TAPVR) is a critical cyanotic congenital heart defect accounting for 1–3% of all congenital cardiac malformations. Survival beyond infancy without surgical correction is exceptional. The coexistence of TAPVR, ostium secundum atrial septal defect (ASD-OS), and cor triatriatum sinister in a single patient represents an extraordinary and scarcely reported anatomical combination. Case Presentation: A 27-year-old male patient R.O. presented to a rheumatology outpatient clinic for musculoskeletal complaints. Clinical examination revealed central cyanosis with peribuccal distribution, digital clubbing, and oxygen desaturation (SpO₂ = 87% on room air). Transthoracic echocardiography performed at CHU Mohammed VI, Marrakech (February 24, 2026), demonstrated: (1) a large 36-mm ASD-OS, (2) a stenosing membrane in the left atrium consistent with cor triatriatum sinister, and (3) anomalous connection of the two left pulmonary veins into a collector draining into the brachiocephalic trunk with stenosis at its junction. Thoracic CT angiography (Hôpital AR-RAZI, February 9, 2026) confirmed ectasia of the superior vena cava draining both right and left pulmonary veins, with the left atrium receiving no venous branches — establishing the diagnosis of supracardiac TAPVR. Moderate pulmonary arterial hypertension was documented (PA trunk 21 mm, Vmax = 2.8 m/s). Total surgical correction was performed on March 27, 2026 (TAPVR repair, ASD closure, and left atrial membrane resection), with a favourable post-operative outcome including resolution of cyanosis, improvement in oxygen saturation, and echocardiographic confirmation of complete ASD closure with no residual shunt. Conclusion: This case represents one of the rarest reported associations of three concurrent congenital cardiac anomalies surviving to adulthood. The large ASD served as a lifesaving hemodynamic bridge. This report emphasises that digital clubbing and cyanosis in any young patient must trigger prompt and systematic cardiac evaluation, regardless of the presenting complaint.