Dextrocardia in Adults with Congenital Heart Disease

Abstract

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.

Keywords: Adult congenital heart disease; Dextrocardia; Mortality.