Sairam Palaparthi 1 , Shastri Ramkinkar 1 , Kishore V K Jayanthi 1 , Nitin K Rao 1 , Girish Warrier 2 , Balaji Srimurugan 3 , B R Jagannath 1 Show Affiliations »
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. © Indian Association of Cardiovascular-Thoracic Surgeons 2022.
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. © Indian Association of Cardiovascular-Thoracic Surgeons 2022.
Entities: Chemical
Keywords:
Anomalous single unilateral pulmonary vein; Heterotaxy; Intrapulmonary vein; Isomerism; Meandering pulmonary vein; Scimitar variant; Total anomalous pulmonary venous connection; Unusual variant TAPVC
Year: 2022
PMID： 35756550 PMCID： PMC9218039 DOI： 10.1007/s12055-021-01290-2
Source DB: PubMed Journal: Indian J Thorac Cardiovasc Surg ISSN： 0970-9134