Iatrogenic coronary sinus diversion to left atrium mimicking paravalvular leak: the importance of documentation

Managing adult patients with congenital heart disease (CHD) presents unique diagnostic as well as therapeutic challenges due to their altered anatomy, complex physiology, and lack of surgical records. We present a case of pseudo-paravalvular leakage after mitral valve (MV) replacement. The patient was a 46-year-old female who presented with severe mitral regurgitation, pulmonary hypertension, and single-vessel coronary artery disease. Per the patient, she was operated on for ventricular septal defect in her childhood. During surgery, it was discovered that the previous surgery was an atrioventricular septal defect (AVSD) repair, and her coronary sinus had been directed into the left atrium. The patient underwent MV replacement and tricuspid valve repair. Her left atrial coronary sinus mimicked a paravalvular leak after weaning from cardiopulmonary bypass (CPB) requiring re-initiation of CPB and arresting the heart. Mitral annular exploration confirmed no residual paravalvular defect, and the jet was persistent at the site of the coronary sinus after weaning from CPB a second time. The patient made an uneventful recovery except for the need for prolonged ventilation due to pulmonary hypertension and chronic obstructive pulmonary disease (COPD). This case highlights the importance of the availability of congenital heart surgery records and comprehensive preoperative imaging prior to reoperation. Our case is an example of anatomical misconception of paravalvular leakage and emphasizes the importance of diligent preoperative and intraoperative imaging to identify the detailed anatomy to prevent unnecessary procedures in adults operated for congenital heart diseases.