Associated congenital heart disease with Hirschsprung's disease: a retrospective cohort study on 2,174 children
ObjectiveTo examine the incidence and phenotypes of congenital heart disease (CHD) in a large cohort of patients with Hirschsprung's disease (HSCR).Study designRetrospective data review of children with HSCR between 2003 and 2020 was conducted at the Provincial Key Laboratory for Structural Bir...
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| Format: | Article |
| Language: | English |
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Frontiers Media S.A.
2023-08-01
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| Series: | Frontiers in Cardiovascular Medicine |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fcvm.2023.1215473/full |
| _version_ | 1797744752646422528 |
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| author | Yujian Wu Yujian Wu Yun Zhu Yun Zhu Xu Zhang Xu Zhang Jinqing Feng Jinqing Feng Huimin Xia Huimin Xia Yan Zhang Yan Zhang Jia Li Jia Li |
| author_facet | Yujian Wu Yujian Wu Yun Zhu Yun Zhu Xu Zhang Xu Zhang Jinqing Feng Jinqing Feng Huimin Xia Huimin Xia Yan Zhang Yan Zhang Jia Li Jia Li |
| author_sort | Yujian Wu |
| collection | DOAJ |
| description | ObjectiveTo examine the incidence and phenotypes of congenital heart disease (CHD) in a large cohort of patients with Hirschsprung's disease (HSCR).Study designRetrospective data review of children with HSCR between 2003 and 2020 was conducted at the Provincial Key Laboratory for Structural Birth Defects in Guangzhou, Guangdong, China. HSCR was confirmed by pathological diagnosis. CHD was defined as a gross structural abnormality of the heart or intrathoracic great vessels that is of functional significance.ResultsA total of 2,174 HSCR patients (84.7% males) were studied and 306 of them underwent echocardiography. Overall, 27 children (1.2%) had associated CHD. Among them, CHDs mostly presented as atrial and ventricular septal defects (n = 5 and 12 respectively) and patent ductus arteriosus (n = 4). Three patients (1.4‰) presented as a severe CHD including complete atrioventricular canal, congenitally corrected transposition of the great arteries and double-outlet of right ventricle. Among 14 patients carrying a chromosomal abnormality, CHD was detected in 4 infants (28.6%), all being mild forms of septal defects.ConclusionsSome new and severe types of CHD were found in patients with HSCR. Patients with syndromic features had higher incidence of CHD. |
| first_indexed | 2024-03-12T15:13:48Z |
| format | Article |
| id | doaj.art-28b4b0106b6140c29488f727ebe30d31 |
| institution | Directory Open Access Journal |
| issn | 2297-055X |
| language | English |
| last_indexed | 2024-03-12T15:13:48Z |
| publishDate | 2023-08-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Cardiovascular Medicine |
| spelling | doaj.art-28b4b0106b6140c29488f727ebe30d312023-08-11T16:21:10ZengFrontiers Media S.A.Frontiers in Cardiovascular Medicine2297-055X2023-08-011010.3389/fcvm.2023.12154731215473Associated congenital heart disease with Hirschsprung's disease: a retrospective cohort study on 2,174 childrenYujian Wu0Yujian Wu1Yun Zhu2Yun Zhu3Xu Zhang4Xu Zhang5Jinqing Feng6Jinqing Feng7Huimin Xia8Huimin Xia9Yan Zhang10Yan Zhang11Jia Li12Jia Li13Department of Pediatric Cardiology, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangzhou, ChinaGuangdong Provincial Key Laboratory of Research in Structural Birth Defect Disease, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangzhou, ChinaGuangdong Provincial Key Laboratory of Research in Structural Birth Defect Disease, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangzhou, ChinaDepartment of Pediatric Surgery, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangzhou, ChinaDepartment of Pediatric Cardiology, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangzhou, ChinaGuangdong Provincial Key Laboratory of Research in Structural Birth Defect Disease, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangzhou, ChinaGuangdong Provincial Key Laboratory of Research in Structural Birth Defect Disease, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangzhou, ChinaClinical Physiology Laboratory, Research Institute of Pediatrics, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangzhou, ChinaGuangdong Provincial Key Laboratory of Research in Structural Birth Defect Disease, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangzhou, ChinaDepartment of Pediatric Surgery, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangzhou, ChinaGuangdong Provincial Key Laboratory of Research in Structural Birth Defect Disease, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangzhou, ChinaDepartment of Pediatric Surgery, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangzhou, ChinaGuangdong Provincial Key Laboratory of Research in Structural Birth Defect Disease, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangzhou, ChinaClinical Physiology Laboratory, Research Institute of Pediatrics, Guangzhou Women and Children’s Medical Center, Guangzhou Medical University, Guangzhou, ChinaObjectiveTo examine the incidence and phenotypes of congenital heart disease (CHD) in a large cohort of patients with Hirschsprung's disease (HSCR).Study designRetrospective data review of children with HSCR between 2003 and 2020 was conducted at the Provincial Key Laboratory for Structural Birth Defects in Guangzhou, Guangdong, China. HSCR was confirmed by pathological diagnosis. CHD was defined as a gross structural abnormality of the heart or intrathoracic great vessels that is of functional significance.ResultsA total of 2,174 HSCR patients (84.7% males) were studied and 306 of them underwent echocardiography. Overall, 27 children (1.2%) had associated CHD. Among them, CHDs mostly presented as atrial and ventricular septal defects (n = 5 and 12 respectively) and patent ductus arteriosus (n = 4). Three patients (1.4‰) presented as a severe CHD including complete atrioventricular canal, congenitally corrected transposition of the great arteries and double-outlet of right ventricle. Among 14 patients carrying a chromosomal abnormality, CHD was detected in 4 infants (28.6%), all being mild forms of septal defects.ConclusionsSome new and severe types of CHD were found in patients with HSCR. Patients with syndromic features had higher incidence of CHD.https://www.frontiersin.org/articles/10.3389/fcvm.2023.1215473/fullHirschsprung’s diseasecongenital heart diseasechromosomal abnormalitycomplete atrioventricular canalcongenitally corrected transposition of the great arteriesdouble-outlet of right ventricle |
| spellingShingle | Yujian Wu Yujian Wu Yun Zhu Yun Zhu Xu Zhang Xu Zhang Jinqing Feng Jinqing Feng Huimin Xia Huimin Xia Yan Zhang Yan Zhang Jia Li Jia Li Associated congenital heart disease with Hirschsprung's disease: a retrospective cohort study on 2,174 children Frontiers in Cardiovascular Medicine Hirschsprung’s disease congenital heart disease chromosomal abnormality complete atrioventricular canal congenitally corrected transposition of the great arteries double-outlet of right ventricle |
| title | Associated congenital heart disease with Hirschsprung's disease: a retrospective cohort study on 2,174 children |
| title_full | Associated congenital heart disease with Hirschsprung's disease: a retrospective cohort study on 2,174 children |
| title_fullStr | Associated congenital heart disease with Hirschsprung's disease: a retrospective cohort study on 2,174 children |
| title_full_unstemmed | Associated congenital heart disease with Hirschsprung's disease: a retrospective cohort study on 2,174 children |
| title_short | Associated congenital heart disease with Hirschsprung's disease: a retrospective cohort study on 2,174 children |
| title_sort | associated congenital heart disease with hirschsprung s disease a retrospective cohort study on 2 174 children |
| topic | Hirschsprung’s disease congenital heart disease chromosomal abnormality complete atrioventricular canal congenitally corrected transposition of the great arteries double-outlet of right ventricle |
| url | https://www.frontiersin.org/articles/10.3389/fcvm.2023.1215473/full |
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