Clinical implications of dextrocardia based on four visceroatrial situs studies

Background: Previous studies on congenital heart diseases (CHD) associated with dextrocardia were based on selective patient databases and did not reflect the full spectrum of dextrocardia in the general population. Additionally, these studies had complex classification and presentation. Nor did the...

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Main Authors: Mao-Sheng Hwang, Ching-Chia Kuo, Chao-Jan Wang, Wen-Jen Su, Jaw-Ji Chu, Hung-Tao Chung, Hsiang-Ju Hsiao, Yi-Jung Chang
Format: Article
Language:English
Published: Elsevier 2024-11-01
Series:Pediatrics and Neonatology
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Online Access:http://www.sciencedirect.com/science/article/pii/S1875957224000457
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author Mao-Sheng Hwang
Ching-Chia Kuo
Chao-Jan Wang
Wen-Jen Su
Jaw-Ji Chu
Hung-Tao Chung
Hsiang-Ju Hsiao
Yi-Jung Chang
author_facet Mao-Sheng Hwang
Ching-Chia Kuo
Chao-Jan Wang
Wen-Jen Su
Jaw-Ji Chu
Hung-Tao Chung
Hsiang-Ju Hsiao
Yi-Jung Chang
author_sort Mao-Sheng Hwang
collection DOAJ
description Background: Previous studies on congenital heart diseases (CHD) associated with dextrocardia were based on selective patient databases and did not reflect the full spectrum of dextrocardia in the general population. Additionally, these studies had complex classification and presentation. Nor did these studies elaborate on the distribution of the associated CHD's complexity, the various segmental connections, and associated CHD among the four visceroatrial situs. Methods: We retrospectively reviewed the medical records of 211 children with primary dextrocardia. We used a segmental approach to diagnose CHD. We then analyzed and compared the distribution of the above-mentioned issues among the four visceroatrial situs. Results: Dextrocardia occurred most commonly with situs inversus (52.6%), followed by situs solitus (28.4%), asplenia (17.1%), and polysplenia (1.9%). Although some patients had a structurally normal heart (22.7%) or they were associated with simple CHD (17.5%), most patients had complex CHD (59.7%) consisting of a single ventricle (34.6%) or conotruncal anomaly (25.1%) (double-outlet right ventricle [7.6%], corrected transposition of the great arteries [6.2%], complete transposition of the great arteries [5.7%], tetralogy of Fallot [4.7%], etc.). Situs inversus or polysplenia had a higher prevalence of a structurally normal heart or associated with simple CHD, two patent atrioventricular (AV) valves connections, and biventricular AV connections. Situs solitus or asplenia had a higher prevalence of associated complex CHD, common AV valve connection, univentricular AV connection, pulmonary outflow tract obstruction, and anomalous pulmonary venous drainage. Conclusion: Our study finds that situs inversus is the most common visceroatrial situs in dextrocardia. Although some patients had a structurally normal heart or were associated with simple CHD, most patients have associated complex CHD consisting of a single ventricle or conotruncal anomaly. Dextrocardia is associated with a higher incidence of complex CHD in situs solitus and asplenia groups than in situs inversus and polysplenia groups.
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spelling doaj-art-dac69ddfe4bd4881b4898e2806d447122025-08-20T02:04:28ZengElsevierPediatrics and Neonatology1875-95722024-11-0165656657010.1016/j.pedneo.2023.10.011Clinical implications of dextrocardia based on four visceroatrial situs studiesMao-Sheng Hwang0Ching-Chia Kuo1Chao-Jan Wang2Wen-Jen Su3Jaw-Ji Chu4Hung-Tao Chung5Hsiang-Ju Hsiao6Yi-Jung Chang7Department of Pediatrics, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan; Corresponding author. Department of Pediatrics, Chang Gung Memorial Hospital, 5-7 Fu-Hsing St, Kwei-Shan, Taoyuan, 333, Taiwan.Department of Pediatrics, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, TaiwanDepartment of Radiology, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, TaiwanDepartment of Pediatrics, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, TaiwanDepartment of Surgery, New Taipei Municipal Tucheng Hospital, Chang Gung Memorial Hospital, Tucheng 236, Taiwan; Chang Gung University College of Medicine, Taoyuan, 333, TaiwanDepartment of Pediatrics, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, TaiwanDepartment of Pediatrics, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, TaiwanDepartment of Pediatrics, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan; Corresponding author. Department of Pediatrics, Chang Gung Memorial Hospital, 5-7 Fu-Hsing St, Kwei-Shan, Taoyuan, 333, Taiwan.Background: Previous studies on congenital heart diseases (CHD) associated with dextrocardia were based on selective patient databases and did not reflect the full spectrum of dextrocardia in the general population. Additionally, these studies had complex classification and presentation. Nor did these studies elaborate on the distribution of the associated CHD's complexity, the various segmental connections, and associated CHD among the four visceroatrial situs. Methods: We retrospectively reviewed the medical records of 211 children with primary dextrocardia. We used a segmental approach to diagnose CHD. We then analyzed and compared the distribution of the above-mentioned issues among the four visceroatrial situs. Results: Dextrocardia occurred most commonly with situs inversus (52.6%), followed by situs solitus (28.4%), asplenia (17.1%), and polysplenia (1.9%). Although some patients had a structurally normal heart (22.7%) or they were associated with simple CHD (17.5%), most patients had complex CHD (59.7%) consisting of a single ventricle (34.6%) or conotruncal anomaly (25.1%) (double-outlet right ventricle [7.6%], corrected transposition of the great arteries [6.2%], complete transposition of the great arteries [5.7%], tetralogy of Fallot [4.7%], etc.). Situs inversus or polysplenia had a higher prevalence of a structurally normal heart or associated with simple CHD, two patent atrioventricular (AV) valves connections, and biventricular AV connections. Situs solitus or asplenia had a higher prevalence of associated complex CHD, common AV valve connection, univentricular AV connection, pulmonary outflow tract obstruction, and anomalous pulmonary venous drainage. Conclusion: Our study finds that situs inversus is the most common visceroatrial situs in dextrocardia. Although some patients had a structurally normal heart or were associated with simple CHD, most patients have associated complex CHD consisting of a single ventricle or conotruncal anomaly. Dextrocardia is associated with a higher incidence of complex CHD in situs solitus and asplenia groups than in situs inversus and polysplenia groups.http://www.sciencedirect.com/science/article/pii/S1875957224000457Cardiac malpositionCongenital heart diseasesDextrocardiaSegmental approachVisceroatrial situs
spellingShingle Mao-Sheng Hwang
Ching-Chia Kuo
Chao-Jan Wang
Wen-Jen Su
Jaw-Ji Chu
Hung-Tao Chung
Hsiang-Ju Hsiao
Yi-Jung Chang
Clinical implications of dextrocardia based on four visceroatrial situs studies
Pediatrics and Neonatology
Cardiac malposition
Congenital heart diseases
Dextrocardia
Segmental approach
Visceroatrial situs
title Clinical implications of dextrocardia based on four visceroatrial situs studies
title_full Clinical implications of dextrocardia based on four visceroatrial situs studies
title_fullStr Clinical implications of dextrocardia based on four visceroatrial situs studies
title_full_unstemmed Clinical implications of dextrocardia based on four visceroatrial situs studies
title_short Clinical implications of dextrocardia based on four visceroatrial situs studies
title_sort clinical implications of dextrocardia based on four visceroatrial situs studies
topic Cardiac malposition
Congenital heart diseases
Dextrocardia
Segmental approach
Visceroatrial situs
url http://www.sciencedirect.com/science/article/pii/S1875957224000457
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