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Published on: February 11, 2022
Clinical implications of dextrocardia based on four visceroatrial situs studies
Mao-Sheng Hwang1, Ching-Chia Kuo1, Chao-Jan Wang2
1Department of Pediatrics, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan, Taiwan.
Insights
Dextrocardia most commonly occurs with situs inversus. Most patients with dextrocardia have complex congenital heart disease (CHD), often a single ventricle or conotruncal anomaly, particularly in situs solitus and asplenia.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Medical Genetics
Background:
- Limited understanding of congenital heart disease (CHD) spectrum in dextrocardia due to selective databases.
- Previous studies lacked comprehensive classification and detailed analysis of CHD complexity and visceral situs distribution.
Purpose of the Study:
- To analyze the spectrum of CHD in children with dextrocardia across different visceroatrial situs.
- To investigate the distribution of CHD complexity, segmental connections, and associated anomalies based on four visceroatrial situs.
Main Methods:
- Retrospective review of 211 pediatric dextrocardia cases.
- Utilized a segmental approach for CHD diagnosis.
- Analyzed and compared CHD characteristics across four visceroatrial situs.
Main Results:
- Dextrocardia prevalence: Situs inversus (52.6%), situs solitus (28.4%), asplenia (17.1%), polysplenia (1.9%).
- Complex CHD (59.7%) was most common, including single ventricle (34.6%) and conotruncal anomalies (25.1%).
- Situs solitus/asplenia showed higher complex CHD rates compared to situs inversus/polysplenia.
Conclusions:
- Situs inversus is the most frequent visceroatrial situs in dextrocardia.
- Complex CHD, particularly single ventricle and conotruncal anomalies, is prevalent in dextrocardia.
- Dextrocardia with situs solitus and asplenia is associated with a greater incidence of complex CHD.
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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