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Malposition of septum primum in isolated dextrocardia: unique and rare form of anomalous pulmonary venous return in association with partial absence of pericardium-case report

BACKGROUND: Total anomalous pulmonary venous return (TAPVR) refers to an anomaly in which all of the pulmonary veins drain directly or indirectly to the systemic venous circulation. However, unusual types constitute approximately 5% or less of TAPVRs and there may be obstruction or discontinuity of...

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Autores principales: Jha, Neerod Kumar, Talo, Haitham, Kiraly, Laszlo, Shah, Nishant, Al Hakami, Aref, Azeez, Zafar Althaf, Bai, Ajitha Kumari Kamala
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2021
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Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8325803/
https://www.ncbi.nlm.nih.gov/pubmed/34332582
http://dx.doi.org/10.1186/s13019-021-01591-y
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author Jha, Neerod Kumar
Talo, Haitham
Kiraly, Laszlo
Shah, Nishant
Al Hakami, Aref
Azeez, Zafar Althaf
Bai, Ajitha Kumari Kamala
author_facet Jha, Neerod Kumar
Talo, Haitham
Kiraly, Laszlo
Shah, Nishant
Al Hakami, Aref
Azeez, Zafar Althaf
Bai, Ajitha Kumari Kamala
author_sort Jha, Neerod Kumar
collection PubMed
description BACKGROUND: Total anomalous pulmonary venous return (TAPVR) refers to an anomaly in which all of the pulmonary veins drain directly or indirectly to the systemic venous circulation. However, unusual types constitute approximately 5% or less of TAPVRs and there may be obstruction or discontinuity of pulmonary vein at various levels. CASE PRESENTATION: A 3-month-old infant was presented to us with history of poor feeding, respiratory distress and desaturations. The routine echocardiographic investigation initially confirmed the diagnosis of an atrial septal defect with dextrocardia. However, due to disproportionate severity of symptoms and congestive heart failure a cardiac computer tomography angiogram was done that revealed a rare finding of connection of pulmonary veins fused with the posterior atrium, but on the rightward side of the deviated atrial septum. Therefore, pulmonary veins entered a sinus that drains directly into the right atrial superior-posterior wall. During surgical repair, we found an area of absent pericardium in the diaphragmatic surface of the heart. The patient underwent total repair of the TAPVR and patch reconstruction of the pericardial defect. The patient is doing well at 6-month follow up. CONCLUSIONS: The septum primum malposition defect resulting in TAPVR is a very rare congenital anomaly that can be rarely seen without any heterotaxy. The anomalous features including absent pericardium and dextrocardia were present in this patient have not been described previously with TAPVR. Therefore, we have hypothesized the embryological correlation of absent pericardium and cardiac malposition in such case. Transthoracic echocardiography with Doppler interrogation is a reliable method for diagnosing this condition. In case of suboptimal echocardiographic image due to cardiac position, unclear anatomy or unexplained symptoms, advanced imaging such as computer tomographic angiography or cardiac magnetic resonance imaging can be very helpful. Preoperative proper diagnosis of this anomaly facilitates successful surgical management with excellent outcome.
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spelling pubmed-83258032021-08-02 Malposition of septum primum in isolated dextrocardia: unique and rare form of anomalous pulmonary venous return in association with partial absence of pericardium-case report Jha, Neerod Kumar Talo, Haitham Kiraly, Laszlo Shah, Nishant Al Hakami, Aref Azeez, Zafar Althaf Bai, Ajitha Kumari Kamala J Cardiothorac Surg Case Report BACKGROUND: Total anomalous pulmonary venous return (TAPVR) refers to an anomaly in which all of the pulmonary veins drain directly or indirectly to the systemic venous circulation. However, unusual types constitute approximately 5% or less of TAPVRs and there may be obstruction or discontinuity of pulmonary vein at various levels. CASE PRESENTATION: A 3-month-old infant was presented to us with history of poor feeding, respiratory distress and desaturations. The routine echocardiographic investigation initially confirmed the diagnosis of an atrial septal defect with dextrocardia. However, due to disproportionate severity of symptoms and congestive heart failure a cardiac computer tomography angiogram was done that revealed a rare finding of connection of pulmonary veins fused with the posterior atrium, but on the rightward side of the deviated atrial septum. Therefore, pulmonary veins entered a sinus that drains directly into the right atrial superior-posterior wall. During surgical repair, we found an area of absent pericardium in the diaphragmatic surface of the heart. The patient underwent total repair of the TAPVR and patch reconstruction of the pericardial defect. The patient is doing well at 6-month follow up. CONCLUSIONS: The septum primum malposition defect resulting in TAPVR is a very rare congenital anomaly that can be rarely seen without any heterotaxy. The anomalous features including absent pericardium and dextrocardia were present in this patient have not been described previously with TAPVR. Therefore, we have hypothesized the embryological correlation of absent pericardium and cardiac malposition in such case. Transthoracic echocardiography with Doppler interrogation is a reliable method for diagnosing this condition. In case of suboptimal echocardiographic image due to cardiac position, unclear anatomy or unexplained symptoms, advanced imaging such as computer tomographic angiography or cardiac magnetic resonance imaging can be very helpful. Preoperative proper diagnosis of this anomaly facilitates successful surgical management with excellent outcome. BioMed Central 2021-07-31 /pmc/articles/PMC8325803/ /pubmed/34332582 http://dx.doi.org/10.1186/s13019-021-01591-y Text en © The Author(s) 2021 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Case Report
Jha, Neerod Kumar
Talo, Haitham
Kiraly, Laszlo
Shah, Nishant
Al Hakami, Aref
Azeez, Zafar Althaf
Bai, Ajitha Kumari Kamala
Malposition of septum primum in isolated dextrocardia: unique and rare form of anomalous pulmonary venous return in association with partial absence of pericardium-case report
title Malposition of septum primum in isolated dextrocardia: unique and rare form of anomalous pulmonary venous return in association with partial absence of pericardium-case report
title_full Malposition of septum primum in isolated dextrocardia: unique and rare form of anomalous pulmonary venous return in association with partial absence of pericardium-case report
title_fullStr Malposition of septum primum in isolated dextrocardia: unique and rare form of anomalous pulmonary venous return in association with partial absence of pericardium-case report
title_full_unstemmed Malposition of septum primum in isolated dextrocardia: unique and rare form of anomalous pulmonary venous return in association with partial absence of pericardium-case report
title_short Malposition of septum primum in isolated dextrocardia: unique and rare form of anomalous pulmonary venous return in association with partial absence of pericardium-case report
title_sort malposition of septum primum in isolated dextrocardia: unique and rare form of anomalous pulmonary venous return in association with partial absence of pericardium-case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8325803/
https://www.ncbi.nlm.nih.gov/pubmed/34332582
http://dx.doi.org/10.1186/s13019-021-01591-y
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