The meandering intrapulmonary total anomalous pulmonary venous channel (MITAPVC)-old wine in new bottle or a new variant?

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 ( = 16) and the other with ( = 14). Of the 20 surgical interventions, only 12 survived, most of them in the isolated group ( = 10). Mortality was due to (4/4), (3/3), and (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.