Background/Objectives: Dextrocardia is rare in the generalpopulation, and may be associated with significant additional cardiacmalformations. We aimed to identify the prevalence and patterns of additionalcardiac defects, as well as the associated long-term morbidity and mortality,in adult patients with dextrocardia, in a specialised Adult Congenital HeartDisease (ACHD) service.
Methods: A retrospective study of patients with dextrocardiamanaged by our tertiary ACHD service, since January 2000, was performed.Medical records were reviewed and the National Death Index was consulted toconfirm 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 atlast 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 singleventricles, of whom five had atrioventricular (AV)-ventriculoarterial (VA)discordance and two had VA discordance only. Four patients with two ventricleshad AV-VA discordance. All patients had at least one additional cardiacmalformation. Fourteen patients (74%) required surgical intervention. Elevenpatients (58%) underwent a Fontan-type operation. Five patients (26%) requiredablation procedures for arrhythmia. One patient had infective endocarditis andtwo 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 complexlesions, which may require multiple surgical and medical interventions. Despitethis, our series demonstrated that patients surviving to adulthood and thenmanaged in an ACHD centre may have good medium-term survival.