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Eisenmenger syndrome with left main compression syndrome: a case report

BACKGROUND: Left main coronary artery disease secondary to pulmonary artery compression related to Eisenmenger syndrome is an under-suspected condition that can cause fatal outcomes if left untreated. It presents with typical angina but is frequently mistaken for pulmonary hypertension (PH) symptoms...

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Autores principales: Cool, Charlotte Johanna, Fathini, Fathy, Adams, Ibnu, Saboe, Aninka, Kusumawardhani, Nuraini Yasmin, Astuti, Astri, Yahya, Achmad Fauzi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8898490/
https://www.ncbi.nlm.nih.gov/pubmed/35247981
http://dx.doi.org/10.1186/s12872-022-02524-w
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author Cool, Charlotte Johanna
Fathini, Fathy
Adams, Ibnu
Saboe, Aninka
Kusumawardhani, Nuraini Yasmin
Astuti, Astri
Yahya, Achmad Fauzi
author_facet Cool, Charlotte Johanna
Fathini, Fathy
Adams, Ibnu
Saboe, Aninka
Kusumawardhani, Nuraini Yasmin
Astuti, Astri
Yahya, Achmad Fauzi
author_sort Cool, Charlotte Johanna
collection PubMed
description BACKGROUND: Left main coronary artery disease secondary to pulmonary artery compression related to Eisenmenger syndrome is an under-suspected condition that can cause fatal outcomes if left untreated. It presents with typical angina but is frequently mistaken for pulmonary hypertension (PH) symptoms. It is now recognized as one of the few important causes of angina in PH. CASE PRESENTATION: A 37-year-old man with a history of unoperated atrial septal defect and Eisenmenger syndrome came to the outpatient department with a chief complaint of angina on exertion. Electrocardiogram showed regular sinus rhythm with right axis deviation, right ventricular hypertrophy, deep T-wave inversion in inferior and anterior leads suggestive of ischemia or strain, and incomplete right bundle branch block. Cardiac CT showed compression of the left main coronary artery due to a dilated main pulmonary artery. Therefore, this patient was diagnosed with Eisenmenger syndrome with left main compression due to dilated pulmonary artery. He was treated successfully with IVUS-guided stent implantation. The patient experienced marked improvement in regular activities, with no recurrence of angina symptoms. Angiography 3 months after the procedure revealed good patency of the stent, without significant stenosis. CONCLUSIONS: Left main coronary artery compression is a complication that should be suspected in patients with Eisenmenger syndrome presenting with angina symptoms. Non-invasive modalities are recommended for diagnostic evaluation, but the gold-standard technique remains coronary angiography. The best treatment is not well-established, with either myocardial revascularization or PH treatment, but a left main coronary artery stenting procedure is considered an ideal emergent treatment to provide a better quality of life for patients in this condition.
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spelling pubmed-88984902022-03-17 Eisenmenger syndrome with left main compression syndrome: a case report Cool, Charlotte Johanna Fathini, Fathy Adams, Ibnu Saboe, Aninka Kusumawardhani, Nuraini Yasmin Astuti, Astri Yahya, Achmad Fauzi BMC Cardiovasc Disord Case Report BACKGROUND: Left main coronary artery disease secondary to pulmonary artery compression related to Eisenmenger syndrome is an under-suspected condition that can cause fatal outcomes if left untreated. It presents with typical angina but is frequently mistaken for pulmonary hypertension (PH) symptoms. It is now recognized as one of the few important causes of angina in PH. CASE PRESENTATION: A 37-year-old man with a history of unoperated atrial septal defect and Eisenmenger syndrome came to the outpatient department with a chief complaint of angina on exertion. Electrocardiogram showed regular sinus rhythm with right axis deviation, right ventricular hypertrophy, deep T-wave inversion in inferior and anterior leads suggestive of ischemia or strain, and incomplete right bundle branch block. Cardiac CT showed compression of the left main coronary artery due to a dilated main pulmonary artery. Therefore, this patient was diagnosed with Eisenmenger syndrome with left main compression due to dilated pulmonary artery. He was treated successfully with IVUS-guided stent implantation. The patient experienced marked improvement in regular activities, with no recurrence of angina symptoms. Angiography 3 months after the procedure revealed good patency of the stent, without significant stenosis. CONCLUSIONS: Left main coronary artery compression is a complication that should be suspected in patients with Eisenmenger syndrome presenting with angina symptoms. Non-invasive modalities are recommended for diagnostic evaluation, but the gold-standard technique remains coronary angiography. The best treatment is not well-established, with either myocardial revascularization or PH treatment, but a left main coronary artery stenting procedure is considered an ideal emergent treatment to provide a better quality of life for patients in this condition. BioMed Central 2022-03-05 /pmc/articles/PMC8898490/ /pubmed/35247981 http://dx.doi.org/10.1186/s12872-022-02524-w Text en © The Author(s) 2022 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
Cool, Charlotte Johanna
Fathini, Fathy
Adams, Ibnu
Saboe, Aninka
Kusumawardhani, Nuraini Yasmin
Astuti, Astri
Yahya, Achmad Fauzi
Eisenmenger syndrome with left main compression syndrome: a case report
title Eisenmenger syndrome with left main compression syndrome: a case report
title_full Eisenmenger syndrome with left main compression syndrome: a case report
title_fullStr Eisenmenger syndrome with left main compression syndrome: a case report
title_full_unstemmed Eisenmenger syndrome with left main compression syndrome: a case report
title_short Eisenmenger syndrome with left main compression syndrome: a case report
title_sort eisenmenger syndrome with left main compression syndrome: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8898490/
https://www.ncbi.nlm.nih.gov/pubmed/35247981
http://dx.doi.org/10.1186/s12872-022-02524-w
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