BACKGROUND/OBJECTIVES: @entity5512 is rare in the general population, and may be associated with significant additional @entity299 . We aimed to identify the prevalence and patterns of additional @entity299 , as well as the associated long-term morbidity and mortality, in adult @entity1 with @entity5512 , in a specialised @entity299 ( @entity299 ) service. METHODS: A retrospective study of @entity1 with @entity5512 managed by our tertiary @entity299 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 @entity299 Service, 19 (0.5%) had @entity5512 . 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 @entity1 (58%) had functional single ventricles, of whom five had atrioventricular (AV)-ventriculoarterial (VA) discordance and two had VA discordance only. Four @entity1 with two ventricles had AV-VA discordance. All @entity1 had at least one additional @entity299 . Fourteen @entity1 (74%) required surgical intervention. Eleven @entity1 (58%) underwent a Fontan-type operation. Five @entity1 (26%) required ablation procedures for @entity943 . One @entity1 had @entity884 and two @entity204 occurred, both in @entity1 who also had AV-VA discordance. CONCLUSION: @entity5512 remains a rare finding in adults, even in a highly select group of @entity1 with known @entity299 . Those with associated @entity4297 are likely to have complex lesions, which may require multiple surgical and medical interventions. Despite this, our series demonstrated that @entity1 surviving to adulthood and then managed in an @entity299 centre may have good medium-term survival.

@entity5512 in Adults with XXXX .

@entity1 :: ('9606', 'Species') :: ['patients', 'patient'] @entity5512 :: ('MESH:D003914', 'Disease') :: ['dextrocardia', 'Dextrocardia'] @entity299 :: ('MESH:D006331', 'Disease') :: ['cardiac defects', 'cardiac malformation', 'congenital heart disease', 'cardiac malformations', 'ACHD', 'Adult Congenital Heart Disease'] @entity943 :: ('MESH:D001145', 'Disease') :: ['arrhythmia'] @entity204 :: ('MESH:D003643', 'Disease') :: ['deaths'] @entity4297 :: ('MESH:D006330', 'Disease') :: ['congenital heart abnormalities'] @entity884 :: ('MESH:D004696', 'Disease') :: ['infective endocarditis']
@entity299 :: (MESH:D006331,Disease) :: ['Congenital Heart Disease']
