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Case Report: An Anomalous Left Hepatic Venous Connection in a Patient With Unexpected Cyanosis

An anomalous left hepatic venous (LHV) connection is an extremely rare cardiac malformation, and left hepatic venous route abnormalities not associated with other cardiac lesions do not require surgical treatment because they are physiologically benign. However, when venous route abnormalities exist...

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Autores principales: Luo, Fanyan, Bu, Haisong
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8570676/
https://www.ncbi.nlm.nih.gov/pubmed/34746066
http://dx.doi.org/10.3389/fped.2021.773935
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author Luo, Fanyan
Bu, Haisong
author_facet Luo, Fanyan
Bu, Haisong
author_sort Luo, Fanyan
collection PubMed
description An anomalous left hepatic venous (LHV) connection is an extremely rare cardiac malformation, and left hepatic venous route abnormalities not associated with other cardiac lesions do not require surgical treatment because they are physiologically benign. However, when venous route abnormalities exist with associated cardiac lesions, the conduct of the cardiac surgical repair must accommodate the abnormal venous anatomy, especially in total cavopulmonary connection patients. Herein, we present a rare case of a 7-year-old Chinese boy about 1 year post bilateral superior vena cava pulmonary anastomosis who presented with severe cyanosis and was referred to our department. However, the patient showed an unexpected gradual decrease in blood oxygen saturation to 60–70% after the extracardiac total cavopulmonary connection (ETCPC) operation. Emergency echocardiography and computed tomography confirmed that the LHV entered the right atrium. Subsequently, the patient undergone completion of a staged TCPC with intra-atrial tunnel technique. This illustrative report highlights the essence of improving the preoperative accurate diagnosis to avoid unplanned reoperation in China, especially for the remote rural areas of eastern countries where the level of health care and services is relatively backward. Failure to identify anomalous LHV connection, in this case, will delay effective treatment past the optimal treatment time.
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spelling pubmed-85706762021-11-06 Case Report: An Anomalous Left Hepatic Venous Connection in a Patient With Unexpected Cyanosis Luo, Fanyan Bu, Haisong Front Pediatr Pediatrics An anomalous left hepatic venous (LHV) connection is an extremely rare cardiac malformation, and left hepatic venous route abnormalities not associated with other cardiac lesions do not require surgical treatment because they are physiologically benign. However, when venous route abnormalities exist with associated cardiac lesions, the conduct of the cardiac surgical repair must accommodate the abnormal venous anatomy, especially in total cavopulmonary connection patients. Herein, we present a rare case of a 7-year-old Chinese boy about 1 year post bilateral superior vena cava pulmonary anastomosis who presented with severe cyanosis and was referred to our department. However, the patient showed an unexpected gradual decrease in blood oxygen saturation to 60–70% after the extracardiac total cavopulmonary connection (ETCPC) operation. Emergency echocardiography and computed tomography confirmed that the LHV entered the right atrium. Subsequently, the patient undergone completion of a staged TCPC with intra-atrial tunnel technique. This illustrative report highlights the essence of improving the preoperative accurate diagnosis to avoid unplanned reoperation in China, especially for the remote rural areas of eastern countries where the level of health care and services is relatively backward. Failure to identify anomalous LHV connection, in this case, will delay effective treatment past the optimal treatment time. Frontiers Media S.A. 2021-10-22 /pmc/articles/PMC8570676/ /pubmed/34746066 http://dx.doi.org/10.3389/fped.2021.773935 Text en Copyright © 2021 Luo and Bu. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Pediatrics
Luo, Fanyan
Bu, Haisong
Case Report: An Anomalous Left Hepatic Venous Connection in a Patient With Unexpected Cyanosis
title Case Report: An Anomalous Left Hepatic Venous Connection in a Patient With Unexpected Cyanosis
title_full Case Report: An Anomalous Left Hepatic Venous Connection in a Patient With Unexpected Cyanosis
title_fullStr Case Report: An Anomalous Left Hepatic Venous Connection in a Patient With Unexpected Cyanosis
title_full_unstemmed Case Report: An Anomalous Left Hepatic Venous Connection in a Patient With Unexpected Cyanosis
title_short Case Report: An Anomalous Left Hepatic Venous Connection in a Patient With Unexpected Cyanosis
title_sort case report: an anomalous left hepatic venous connection in a patient with unexpected cyanosis
topic Pediatrics
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8570676/
https://www.ncbi.nlm.nih.gov/pubmed/34746066
http://dx.doi.org/10.3389/fped.2021.773935
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