Improved long-term outcomes in double-inlet left ventricle and tricuspid atresia with transposed great arteries: systemic outflow tract obstruction present at birth defines long-term outcome.
OBJECTIVE
In 2 subtypes of functional single ventricle, double inlet left ventricle (DILV) and tricuspid atresia with transposed great arteries (TA-TGA), systemic output passes through an outflow chamber before entering the aorta. Intracardiac obstruction to this pathway causing systemic outflow tract obstruction (SOTO) may be present at birth or develop over time. Long-term survival after Fontan has not been defined. We defined outcomes utilizing records from 2 centres that were cross-checked with data from a bi-national Fontan Registry for completeness and accuracy.


METHODS
Two hundred and eleven patients were identified, 59 TA-TGA,152 DILV. Median follow-up was 17 years (range 4 days to 49.8 years). The Kaplan-Meier method was used for all of the time to event analyses and the log-rank test was used to compare the time-to-events. Cox proportional hazard models were used to test the association between potential predictors and time-to-event end-points.


RESULTS
TA-TGA had reduced survival compared to DILV (cumulative risk of death 28.8% vs 11%, hazard ratio (HR) 3.1 (95% confidence interval (CI) 1.6-6.1), P  = 0.001). In both groups, SOTO at birth carried a worse prognosis HR 3.54 (1.36-9.2, P  = 0.01). SOTO was not more common in either morphology at birth ( P  = 0.20). Periprocedural mortality accounted for 40% of deaths. Fontan was achieved in 82%, DILV were more likely to achieve Fontan than TA-TGA (91% vs 60%, P <0.001). After Fontan there were 9 deaths (4%) with no difference according to morphology.


CONCLUSIONS
Patients with TA-TGA have poorer outcomes than those with DILV, affecting survival and likelihood of achieving Fontan. SOTO at birth carries a high risk of mortality suggesting that, when present, initial surgical management should address this.
Heart Centre for Children, The Children's Hospital at Westmead, Sydney Children's Hospital Network, Westmead, NSW, Australia.|Amsterdam Medical College, The Netherlands.
Lotte C
LC
Franken
Heart Centre for Children, The Children's Hospital at Westmead, Sydney Children's Hospital Network, Westmead, NSW, Australia.|Amsterdam Medical College, The Netherlands.
Manouk
M
Admiraal
Heart Centre for Children, The Children's Hospital at Westmead, Sydney Children's Hospital Network, Westmead, NSW, Australia.|Australian and New Zealand Fontan Registry.
Charlotte E
CE
Verrall
Murdoch Children's Research Institute, Melbourne, VIC, Australia.
Diana
D
Zannino
Heart Centre for Children, The Children's Hospital at Westmead, Sydney Children's Hospital Network, Westmead, NSW, Australia.|Australian and New Zealand Fontan Registry.|Discipline of Child and Adolescent Health, Sydney Medical School, University of Sydney, NSW, Australia.
Julian G
JG
Ayer
Australian and New Zealand Fontan Registry.|Murdoch Children's Research Institute, Melbourne, VIC, Australia.|Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, VIC, Australia.|Department of Paediatrics, Faculty of Medicine, The University of Melbourne, Parkville, VIC, Australia.
Ajay J
AJ
Iyengar
Heart Centre for Children, The Children's Hospital at Westmead, Sydney Children's Hospital Network, Westmead, NSW, Australia.
Andrew D
AD
Cole
Heart Centre for Children, The Children's Hospital at Westmead, Sydney Children's Hospital Network, Westmead, NSW, Australia.|Australian and New Zealand Fontan Registry.|Discipline of Child and Adolescent Health, Sydney Medical School, University of Sydney, NSW, Australia.
Gary F
GF
Sholler
Australian and New Zealand Fontan Registry.|Murdoch Children's Research Institute, Melbourne, VIC, Australia.|Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, VIC, Australia.|Department of Paediatrics, Faculty of Medicine, The University of Melbourne, Parkville, VIC, Australia.
Yves
Y
D'Udekem
Heart Centre for Children, The Children's Hospital at Westmead, Sydney Children's Hospital Network, Westmead, NSW, Australia.|Murdoch Children's Research Institute, Melbourne, VIC, Australia.|Discipline of Child and Adolescent Health, Sydney Medical School, University of Sydney, NSW, Australia.
David S
DS
Winlaw
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D018729:Fontan Procedure; D006352:Heart Ventricles / Q000601:surgery*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D014188:Transposition of Great Vessels / Q000453:epidemiology / Q000401:mortality* / Q000601:surgery*; D016896:Treatment Outcome; D018785:Tricuspid Atresia / Q000453:epidemiology / Q000401:mortality* / Q000601:surgery*; D055815:Young Adult
 Congenital heart disease ; Great vessel anomalies ; Long-term outcomes; Transposition ; Univentricular heart 