Rare Adult Survival with TAPVR, ASD, and Cor Triatriatum Sinister: A Case Report
Asmaa Elfathi *
Department of Cardiology, Arrazi Hospital, Mohammed VI University Hospital Center (CHU), Marrakech, Morocco.
Mariam Ouaziz
Department of Cardiology, Arrazi Hospital, Mohammed VI University Hospital Center (CHU), Marrakech, Morocco and Faculty of Medicine and Pharmacy, Cadi Ayyad University, Marrakech, Morocco
Imane Chadbellah
Department of Cardiology, Arrazi Hospital, Mohammed VI University Hospital Center (CHU), Marrakech, Morocco and Faculty of Medicine and Pharmacy, Cadi Ayyad University, Marrakech, Morocco.
Sana Nehame
Department of Cardiology, Arrazi Hospital, Mohammed VI University Hospital Center (CHU), Marrakech, Morocco and Faculty of Medicine and Pharmacy, Cadi Ayyad University, Marrakech, Morocco.
Joumana El Masrioui
Department of Cardiology, Arrazi Hospital, Mohammed VI University Hospital Center (CHU), Marrakech, Morocco and Faculty of Medicine and Pharmacy, Cadi Ayyad University, Marrakech, Morocco.
Saloua El karimi
Department of Cardiology, Arrazi Hospital, Mohammed VI University Hospital Center (CHU), Marrakech, Morocco and Faculty of Medicine and Pharmacy, Cadi Ayyad University, Marrakech, Morocco.
Mustapha El Hattaoui
Department of Cardiology, Arrazi Hospital, Mohammed VI University Hospital Center (CHU), Marrakech, Morocco and Faculty of Medicine and Pharmacy, Cadi Ayyad University, Marrakech, Morocco.
*Author to whom correspondence should be addressed.
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.
Keywords: Total anomalous pulmonary venous return, Cor triatriatum sinister, Atrial septal defect, Congenital heart disease, Adults, Cyanosis, Digital clubbing, Pulmonary hypertension