RESUMO
What do we call a vascular structure that is in the left pneumopericardial space, drains systemic venous blood, and the total pulmonary venous return, into the right atrium, through a dilated coronary sinus (CS)? Can we preserve the CS drainage while correcting the total anomalous pulmonary venous connection?
RESUMO
Background: Total anomalous pulmonary venous connection (TAPVC), with an intrapulmonary segment (IPV), a meandering abnormally located confluence and obligatory drainage of one lung into the other before entering the systemic circulation, is a rare anomaly and we term it as the meandering intrapulmonary TAPVC (MITAPVC). Material and methods: We report five patients with an unusual variation of the TAPVC channel. A review of literature was done to identify this association of TAPVC with an intrapulmonary vein and absence of a confluence in its usual location. Results: In our study, 4 patients with neo-confluence creation had excellent outcome while one with partial correction required catheter-based intervention, but succumbed to persistent pulmonary hypertension refractory to therapy. A literature search showed 25 additional such patients. Two groups were noted, one with isolated lesions (N = 16) and the other with heterotaxy or complex intracardiac lesions (N = 14). Of the 20 surgical interventions, only 12 survived, most of them in the isolated group (N = 10). Mortality was due to incomplete surgery (4/4), inappropriate surgery (3/3), and complete and appropriate surgery (1/11) respectively. Conclusion: The MITAPVC is often associated with heterotaxy and complex lesions. However, the isolated version is being increasingly recognised. Non-recognition or inappropriate surgical correction of MITAPVC is associated with fatal outcomes. Evaluation by a computerised tomography (CT) scan, meticulous dissection and demonstration of the entire channel, creation of a neo-confluence and appropriate palliation for the heterotaxy is the key to ensure good outcome. This is not a new entity, but deserves a separate subclassification under TAPVC. Supplementary Information: The online version contains supplementary material available at 10.1007/s12055-021-01290-2.
RESUMO
Background: Surgical correction of total anomalous pulmonary venous connection (TAPVC) remains associated with significant mortality despite advances in intra-operative and postoperative management. We retrospectively analyzed 492 consecutive TAPVC patients with biventricular physiology, who were operated at our centre, with regard to predictors of mortality, morbidity, and intermediate-term outcomes. Materials and Methods: A total of 492 TAPVC patients with biventricular physiology were operated at our centre from August 2009 to November 2019. Their medical records were reviewed and were followed up during March-April 2020 for any symptoms of cardiac disease. Results: Of 492, 302 (61.38%) were healthy at follow-up, 29 (5.89%) had postoperative mortality, 23 (4.67%) had mortality during the follow-up period, and 138 (28.05%) were lost to follow up. Age <1 month and weight <2.5 kg were associated with higher mortality with odds ratios (OR) of 6.37 and 5.56, respectively. There was no difference in mortality in different types of TAPVC. Obstructed TAPVC was associated with higher mortality with OR of 3.05. Acute kidney injury requiring peritoneal dialysis and sepsis were associated with higher mortality with ORs of 10.17 and 3.29, respectively. All follow-up mortality occurred in <1 year from the index operation. Anastomotic gradients were significantly higher in patients who died. Conclusions: Although peri-operative TAPVC mortality has reduced, mortality on follow-up continues to occur and is partly due to the obstruction of pulmonary venous pathway. Meticulous follow-up holds the key in further reducing the mortality. Larger studies are needed for the identification of risk factors for pulmonary venous obstruction and its preventive strategies.