Abnormalities of Cardiac Situs and Heart Disease Diagnosed by Echocardiography in Patients with Biliary Atresia

Abstract Background: Left isomerism (LI) is a common finding in patients with biliary atresia (BA), and it can be identified by echocardiography. Several comorbidities may be present in patients with LI, including heart disease. Objective: To investigate the prevalence of LI and heart disease in children (< 18 years of age) with BA followed-up at Hospital das Clínicas, UFMG. Methods: This is a cross-sectional study involving patients diagnosed with BA between February 2016 and April 2020 who underwent transthoracic echocardiography and, in case of situs abnormalities, also electrocardiography. Results: Our study recruited 58 patients (mean age: 3.08 years; female/male ratio: 1.5:1). The general prevalence of situs abnormalities was 8.6% (5/58) and the most common one was LI (4/5 or 80%). One patient had situs inversus. Among patients with situs abnormalities, the general prevalence of heart disease was 80% (4/5), apart from anomalies of the inferior vena cava), with pulmonary valve stenosis (PVS) as the only change seen (75% of mild forms and 25% of moderate forms). Among patients with situs abnormalities, the prevalence of rhythm changes was 80% (4/5), and low atrial rhythm was the most common finding (3/4 or 75%). Conclusion: The prevalence of situs abnormalities in our sample was similar to that described in the literature. We observed an exclusive prevalence of PVS and a high prevalence of rhythm changes among patients with LI. Although the diagnosis of isomerism does not initially add much cardiovascular risk to the sample, possible late deterioration should be considered.

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Main Authors: Tonelli,Henrique de Assis Fonseca, Meira,Zilda Maria Alves, Castilho,Sandra Regina Tolentino, Guimarães,Adriana Furletti Machado, Queiroz,Tháis Costa Nascentes, Ferreira,Alexandre Rodrigues
Format: Digital revista
Language:English
Published: Sociedade Brasileira de Cardiologia 2022
Online Access:http://old.scielo.br/scielo.php?script=sci_arttext&pid=S2359-56472022000500657
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spelling oai:scielo:S2359-564720220005006572022-09-08Abnormalities of Cardiac Situs and Heart Disease Diagnosed by Echocardiography in Patients with Biliary AtresiaTonelli,Henrique de Assis FonsecaMeira,Zilda Maria AlvesCastilho,Sandra Regina TolentinoGuimarães,Adriana Furletti MachadoQueiroz,Tháis Costa NascentesFerreira,Alexandre Rodrigues Heterotaxy Syndrome Heart diseases Biliary atresia Abstract Background: Left isomerism (LI) is a common finding in patients with biliary atresia (BA), and it can be identified by echocardiography. Several comorbidities may be present in patients with LI, including heart disease. Objective: To investigate the prevalence of LI and heart disease in children (< 18 years of age) with BA followed-up at Hospital das Clínicas, UFMG. Methods: This is a cross-sectional study involving patients diagnosed with BA between February 2016 and April 2020 who underwent transthoracic echocardiography and, in case of situs abnormalities, also electrocardiography. Results: Our study recruited 58 patients (mean age: 3.08 years; female/male ratio: 1.5:1). The general prevalence of situs abnormalities was 8.6% (5/58) and the most common one was LI (4/5 or 80%). One patient had situs inversus. Among patients with situs abnormalities, the general prevalence of heart disease was 80% (4/5), apart from anomalies of the inferior vena cava), with pulmonary valve stenosis (PVS) as the only change seen (75% of mild forms and 25% of moderate forms). Among patients with situs abnormalities, the prevalence of rhythm changes was 80% (4/5), and low atrial rhythm was the most common finding (3/4 or 75%). Conclusion: The prevalence of situs abnormalities in our sample was similar to that described in the literature. We observed an exclusive prevalence of PVS and a high prevalence of rhythm changes among patients with LI. Although the diagnosis of isomerism does not initially add much cardiovascular risk to the sample, possible late deterioration should be considered.info:eu-repo/semantics/openAccessSociedade Brasileira de CardiologiaInternational Journal of Cardiovascular Sciences v.35 n.5 20222022-10-01info:eu-repo/semantics/articletext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S2359-56472022000500657en10.36660/ijcs.20210207
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language English
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author Tonelli,Henrique de Assis Fonseca
Meira,Zilda Maria Alves
Castilho,Sandra Regina Tolentino
Guimarães,Adriana Furletti Machado
Queiroz,Tháis Costa Nascentes
Ferreira,Alexandre Rodrigues
spellingShingle Tonelli,Henrique de Assis Fonseca
Meira,Zilda Maria Alves
Castilho,Sandra Regina Tolentino
Guimarães,Adriana Furletti Machado
Queiroz,Tháis Costa Nascentes
Ferreira,Alexandre Rodrigues
Abnormalities of Cardiac Situs and Heart Disease Diagnosed by Echocardiography in Patients with Biliary Atresia
author_facet Tonelli,Henrique de Assis Fonseca
Meira,Zilda Maria Alves
Castilho,Sandra Regina Tolentino
Guimarães,Adriana Furletti Machado
Queiroz,Tháis Costa Nascentes
Ferreira,Alexandre Rodrigues
author_sort Tonelli,Henrique de Assis Fonseca
title Abnormalities of Cardiac Situs and Heart Disease Diagnosed by Echocardiography in Patients with Biliary Atresia
title_short Abnormalities of Cardiac Situs and Heart Disease Diagnosed by Echocardiography in Patients with Biliary Atresia
title_full Abnormalities of Cardiac Situs and Heart Disease Diagnosed by Echocardiography in Patients with Biliary Atresia
title_fullStr Abnormalities of Cardiac Situs and Heart Disease Diagnosed by Echocardiography in Patients with Biliary Atresia
title_full_unstemmed Abnormalities of Cardiac Situs and Heart Disease Diagnosed by Echocardiography in Patients with Biliary Atresia
title_sort abnormalities of cardiac situs and heart disease diagnosed by echocardiography in patients with biliary atresia
description Abstract Background: Left isomerism (LI) is a common finding in patients with biliary atresia (BA), and it can be identified by echocardiography. Several comorbidities may be present in patients with LI, including heart disease. Objective: To investigate the prevalence of LI and heart disease in children (< 18 years of age) with BA followed-up at Hospital das Clínicas, UFMG. Methods: This is a cross-sectional study involving patients diagnosed with BA between February 2016 and April 2020 who underwent transthoracic echocardiography and, in case of situs abnormalities, also electrocardiography. Results: Our study recruited 58 patients (mean age: 3.08 years; female/male ratio: 1.5:1). The general prevalence of situs abnormalities was 8.6% (5/58) and the most common one was LI (4/5 or 80%). One patient had situs inversus. Among patients with situs abnormalities, the general prevalence of heart disease was 80% (4/5), apart from anomalies of the inferior vena cava), with pulmonary valve stenosis (PVS) as the only change seen (75% of mild forms and 25% of moderate forms). Among patients with situs abnormalities, the prevalence of rhythm changes was 80% (4/5), and low atrial rhythm was the most common finding (3/4 or 75%). Conclusion: The prevalence of situs abnormalities in our sample was similar to that described in the literature. We observed an exclusive prevalence of PVS and a high prevalence of rhythm changes among patients with LI. Although the diagnosis of isomerism does not initially add much cardiovascular risk to the sample, possible late deterioration should be considered.
publisher Sociedade Brasileira de Cardiologia
publishDate 2022
url http://old.scielo.br/scielo.php?script=sci_arttext&pid=S2359-56472022000500657
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