Successful Surgical Correction of Coronary Sinus Total Anomalous Pulmonary Venous Connection Without rFVIIa Intervention in Congenital Factor VII Deficiency

Total anomalous pulmonary venous connection (TAPVC) is a rare cyanotic congenital heart defect where all pulmonary veins drain into systemic veins rather than the left atrium, impairing oxygenation and risking heart failure or pulmonary hypertension, thus necessitating surgery; when coexisting with Factor VII deficiency—an autosomal recessive disorder impairing the extrinsic coagulation pathway—surgical bleeding risk increases, requiring multidisciplinary planning. We report a 7-year-old girl from Jammu and Kashmir, India, presenting with cyanosis during crying, where echocardiography and cardiac catheterization confirmed coronary sinus-type TAPVC and a 14 mm ostium secundum atrial septal defect (OS-ASD) that partially relieved right atrial pressure; she had congenital Factor VII deficiency (14.8% activity preoperatively), demanding vigilant coagulopathy monitoring. In 2024, she developed a brain abscess and left forearm osteomyelitis, treated from February 18 to April 1, with burrhole drainage for the abscess revealing the Factor VII deficiency via coagulopathy workup. Readmitted on October 28, 2025, she underwent surgery on November 26, 2025, involving unroofing of the coronary sinus to redirect pulmonary veins to the left atrium and OS-ASD patch closure via midline sternotomy under cardiopulmonary bypass; prothrombin time and fibrinogen levels remained stable intra- and postoperatively, eliminating the need for recombinant Factor VIIa (rFVIIa), with postoperative Factor VII activity at 13%. She tolerated weaning from inotropes and ventilation without complications, had sutures removed, and was discharged on December 7, 2025, on enalapril for afterload reduction, sildenafil for pulmonary vasodilation, furosemide for diuresis, and amiodarone for rhythm control. This case illustrates successful management of moderate Factor VII deficiency in complex pediatric cardiac surgery without hemostatic prophylaxis, given minimal bleeding and rigorous monitoring, providing insights for resource-limited settings and emphasizing cardiothoracic-haematology collaboration for optimal outcomes.