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Published on: February 11, 2022
Dextrocardia in Adults with Congenital Heart Disease
Sophie Offen1, Dan Jackson2, Carla Canniffe2
1Department of Cardiology, Royal Prince Alfred Hospital, Sydney, Australia; Sydney Medical School, University of Sydney, Sydney, Australia.
Insights
Dextrocardia is rare in adults, often accompanied by complex heart defects requiring interventions. Patients managed in Adult Congenital Heart Disease (ACHD) services show good medium-term survival despite associated cardiac malformations.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Adult Congenital Heart Disease
Background:
- Dextrocardia is a rare cardiac malformation with potential for significant associated defects.
- Understanding the spectrum of these defects and their outcomes in adults is crucial for specialized care.
Purpose of the Study:
- To determine the prevalence and patterns of cardiac defects in adults with dextrocardia.
- To evaluate the long-term morbidity and mortality in this patient population within an Adult Congenital Heart Disease (ACHD) service.
Main Methods:
- Retrospective study of adult patients with dextrocardia managed at a tertiary ACHD service since January 2000.
- Review of medical records and consultation of the National Death Index for survival status.
Main Results:
- Dextrocardia identified in 0.5% of 3698 adults in the ACHD service.
- All patients had additional cardiac malformations, with 58% having functional single ventricles and 74% requiring surgery.
- Two deaths occurred, both in patients with atrioventricular (AV)-ventriculoarterial (VA) discordance.
Conclusions:
- Dextrocardia in adults, even within ACHD services, is rare and typically involves complex congenital heart disease.
- These patients often require multiple interventions, but good medium-term survival is achievable with specialized management.
Background/Objectives:
Dextrocardia is rare in the general population, and may be associated with significant additional cardiac malformations. We aimed to identify the prevalence and patterns of additional cardiac defects, as well as the associated long-term morbidity and mortality, in adult patients with dextrocardia, in a specialised Adult Congenital Heart Disease (ACHD) service.
Methods:
A retrospective study of patients with dextrocardia managed by our tertiary ACHD service, since January 2000, was performed. Medical records were reviewed and the National Death Index was consulted to confirm survival status.
Results:
Of 3698 adults in our ACHD Service, 19 (0.5%) had dextrocardia. Mean follow-up duration was 7±7.5 years. The mean age at last review was 36.8±10.5 years (range 20-63 years). Situs was solitus in 14 (74%) and inversus in five (26%). Eleven patients (58%) had functional single ventricles, of whom five had atrioventricular (AV)-ventriculoarterial (VA) discordance and two had VA discordance only. Four patients with two ventricles had AV-VA discordance. All patients had at least one additional cardiac malformation. Fourteen patients (74%) required surgical intervention. Eleven patients (58%) underwent a Fontan-type operation. Five patients (26%) required ablation procedures for arrhythmia. One patient had infective endocarditis and two deaths occurred, both in patients who also had AV-VA discordance.
Conclusion:
Dextrocardia remains a rare finding in adults, even in a highly select group of patients with known congenital heart disease. Those with associated congenital heart abnormalities are likely to have complex lesions, which may require multiple surgical and medical interventions. Despite this, our series demonstrated that patients surviving to adulthood and then managed in an ACHD centre may have good medium-term survival.
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