Clinical implications of dextrocardia based on four visceroatrial situs studies

Mao-Sheng Hwang; Ching-Chia Kuo; Chao-Jan Wang; Wen-Jen Su; Jaw-Ji Chu; Hung-Tao Chung; Hsiang-Ju Hsiao; Yi-Jung Chang

doi:10.1016/j.pedneo.2023.10.011

Clinical implications of dextrocardia based on four visceroatrial situs studies

Pediatr Neonatol. 2024 Apr 23:S1875-9572(24)00045-7. doi: 10.1016/j.pedneo.2023.10.011. Online ahead of print.

Authors

Mao-Sheng Hwang¹, Ching-Chia Kuo², Chao-Jan Wang³, Wen-Jen Su², Jaw-Ji Chu⁴, Hung-Tao Chung², Hsiang-Ju Hsiao², Yi-Jung Chang⁵

Affiliations

¹ Department of Pediatrics, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan. Electronic address: hms3013@cgmh.org.tw.
² Department of Pediatrics, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan.
³ Department of Radiology, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan.
⁴ Department of Surgery, New Taipei Municipal Tucheng Hospital, Chang Gung Memorial Hospital, Tucheng 236, Taiwan; Chang Gung University College of Medicine, Taoyuan, 333, Taiwan.
⁵ Department of Pediatrics, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan. Electronic address: r64321@cgmh.org.tw.

PMID: 38692948
DOI: 10.1016/j.pedneo.2023.10.011

Abstract

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.

Keywords: Cardiac malposition; Congenital heart diseases; Dextrocardia; Segmental approach; Visceroatrial situs.