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The meandering intrapulmonary total anomalous pulmonary venous channel (MITAPVC)-old wine in new bottle or a new variant?
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ABSTRACT: 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.
SUBMITTER: Palaparthi S
PROVIDER: S-EPMC9218039 | biostudies-literature | 2022 Jul
REPOSITORIES: biostudies-literature
Publications
The meandering intrapulmonary total anomalous pulmonary venous channel (MITAPVC)-old wine in new bottle or a new variant?
Indian journal of thoracic and cardiovascular surgery 20220323 4
<h4>Background</h4>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).<h4>Material and methods</h4>We report five patients with an unusual variation of the TAPVC channel. A review of literature was done to identify this association of TAPVC w ...[more]