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Anomalous right coronary artery originating from the aorta: a series of nine pediatric cases
BACKGROUND: To investigate the clinical manifestations, prognosis, and possibly related genes of anomalous right coronary artery originating from the aorta (ARCA-L) in children. METHODS: This case series study included pediatric patients diagnosed with ARCA-L at the Department of Cardiology in Beiji...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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BioMed Central
2023
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10617201/ https://www.ncbi.nlm.nih.gov/pubmed/37907926 http://dx.doi.org/10.1186/s12887-023-04377-4 |
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author | Na, Jia Chen, Xi Zhen, Zhen Gao, Lu Yuan, Yue |
author_facet | Na, Jia Chen, Xi Zhen, Zhen Gao, Lu Yuan, Yue |
author_sort | Na, Jia |
collection | PubMed |
description | BACKGROUND: To investigate the clinical manifestations, prognosis, and possibly related genes of anomalous right coronary artery originating from the aorta (ARCA-L) in children. METHODS: This case series study included pediatric patients diagnosed with ARCA-L at the Department of Cardiology in Beijing Children’s Hospital affiliated to Capital Medical University, between January 2017 and December 2019. RESULTS: Nine pediatric patients (aged 3 months to 12 years, 4 boys) were included. Two cases presented with cardiac insufficiency as their primary manifestation, while the remaining seven had post-infection or post-exercise symptoms such as chest pain, chest tightness, long exhalation, lack of strength, and dizziness. Six patients displayed varying degrees of ST-T changes on the electrocardiograph, while two patients had a reduced left ventricular ejection fraction (LVEF) of 20-32% according to echocardiography. Multislice computed tomographic angiography confirmed the presence of ARCA-L in all patients. One patient underwent the unroofing technique. The remaining eight received conservative treatment. After a follow-up of 2–64 months, eight children had a good prognosis and survived. One child experienced sudden death due to aggravated heart failure. Whole exome sequencing revealed that one child tested negative, one had mutations in the RYR2 and LDB3 genes, and the remaining four patients had a mutation in the GDF1, LRP6, MEF2A, and KALRN genes, respectively. CONCLUSIONS: ARCA-L in children might have a wide variation in clinical manifestations and a risk of sudden death. The occurrence of the disease might be associated with genetic defects. |
format | Online Article Text |
id | pubmed-10617201 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-106172012023-11-01 Anomalous right coronary artery originating from the aorta: a series of nine pediatric cases Na, Jia Chen, Xi Zhen, Zhen Gao, Lu Yuan, Yue BMC Pediatr Research BACKGROUND: To investigate the clinical manifestations, prognosis, and possibly related genes of anomalous right coronary artery originating from the aorta (ARCA-L) in children. METHODS: This case series study included pediatric patients diagnosed with ARCA-L at the Department of Cardiology in Beijing Children’s Hospital affiliated to Capital Medical University, between January 2017 and December 2019. RESULTS: Nine pediatric patients (aged 3 months to 12 years, 4 boys) were included. Two cases presented with cardiac insufficiency as their primary manifestation, while the remaining seven had post-infection or post-exercise symptoms such as chest pain, chest tightness, long exhalation, lack of strength, and dizziness. Six patients displayed varying degrees of ST-T changes on the electrocardiograph, while two patients had a reduced left ventricular ejection fraction (LVEF) of 20-32% according to echocardiography. Multislice computed tomographic angiography confirmed the presence of ARCA-L in all patients. One patient underwent the unroofing technique. The remaining eight received conservative treatment. After a follow-up of 2–64 months, eight children had a good prognosis and survived. One child experienced sudden death due to aggravated heart failure. Whole exome sequencing revealed that one child tested negative, one had mutations in the RYR2 and LDB3 genes, and the remaining four patients had a mutation in the GDF1, LRP6, MEF2A, and KALRN genes, respectively. CONCLUSIONS: ARCA-L in children might have a wide variation in clinical manifestations and a risk of sudden death. The occurrence of the disease might be associated with genetic defects. BioMed Central 2023-10-31 /pmc/articles/PMC10617201/ /pubmed/37907926 http://dx.doi.org/10.1186/s12887-023-04377-4 Text en © The Author(s) 2023 https://creativecommons.org/licenses/by/4.0/Open Access This 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 | Research Na, Jia Chen, Xi Zhen, Zhen Gao, Lu Yuan, Yue Anomalous right coronary artery originating from the aorta: a series of nine pediatric cases |
title | Anomalous right coronary artery originating from the aorta: a series of nine pediatric cases |
title_full | Anomalous right coronary artery originating from the aorta: a series of nine pediatric cases |
title_fullStr | Anomalous right coronary artery originating from the aorta: a series of nine pediatric cases |
title_full_unstemmed | Anomalous right coronary artery originating from the aorta: a series of nine pediatric cases |
title_short | Anomalous right coronary artery originating from the aorta: a series of nine pediatric cases |
title_sort | anomalous right coronary artery originating from the aorta: a series of nine pediatric cases |
topic | Research |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10617201/ https://www.ncbi.nlm.nih.gov/pubmed/37907926 http://dx.doi.org/10.1186/s12887-023-04377-4 |
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