Survival into adulthood of patients with atrial isomerism undergoing cardiac surgery.
OBJECTIVE
To identify determinants of adverse outcomes in patients with atrial isomerism.


METHODS
Determinants of survival were analyzed for the group as a whole as well as separately for left and right atrial isomerism.


RESULTS
There were 78 cases with right and 104 with left atrial isomerism. Nineteen patients were not offered surgery; 49 (47%) of those with left atrial isomerism and 60 (77%) with right atrial isomerism were directed to single ventricle palliation. A total of 67 patients died. Survival to 25 years was 62% (95% confidence interval [CI], 53%-69%). Independent predictors of mortality were obstructed total anomalous pulmonary venous drainage (P < .001; hazard ratio [HR], 5.2; 95% CI, 2.7-9.7) and asplenia (P = .008; HR, 2; 95% CI, 1.2-3.3). There was no evidence that patients born in the recent era had improved survival (P = .47; HR, 1.1; 95% CI, 0.8-1.5). Survival was better for patients with left than right atrial isomerism: 18 years survival 74% (95% CI, 63%-82%) versus 50% (95% CI, 38%-60%). Independent predictors of mortality for patients with left atrial isomerism were dextrocardia (P = .009; HR, 3.0; 95% CI, 1.3-6.7) and pulmonary stenosis (P = .042; HR, 0.3; 95% CI, 0.1-0.9) and patients with right atrial isomerism, biventricular repair (P < .001; HR, 6.0; 95% CI, 2.8-13), and obstructed total anomalous pulmonary venous drainage (P < .001; HR, 4.2; 95% CI, 2.0-8.6).


CONCLUSIONS
A significant proportion of patients with isomerism still die before reaching adulthood. Only a fraction of those with obstructed pulmonary veins survive. Having biventricular repair does not confer a survival advantage to those born with right atrial isomerism.
Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia.
Jayapadman
J
Bhaskar
The Clinical Epidemiology and Biostatistics Unit, Murdoch Children's Research Institute, Melbourne, Australia; Department of Mathematics and Statistics, La Trobe University, Melbourne, Australia.
John C
JC
Galati
Department of Cardiology, Royal Children's Hospital, Melbourne, Australia.
Paul
P
Brooks
Department of Pediatric Cardiac Surgery, S. Orsola-Malpighi Hospital, Bologna, Italy.
Guido
G
Oppido
Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia; Department of Pediatrics, University of Melbourne, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia.
Igor E
IE
Konstantinov
Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia; Department of Pediatrics, University of Melbourne, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia.
Christian P
CP
Brizard
Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia; Department of Pediatrics, University of Melbourne, Melbourne, Australia; Murdoch Children's Research Institute, Melbourne, Australia. Electronic address: yves.dudekem@rch.org.au.
Yves
Y
d'Udekem
D000015:Abnormalities, Multiple; D000328:Adult; D006348:Cardiac Surgical Procedures / Q000009:adverse effects / Q000401:mortality*; D005260:Female; D059446:Heterotaxy Syndrome / Q000175:diagnosis / Q000401:mortality / Q000601:surgery*; D006776:Hospitals, Pediatric; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D016017:Odds Ratio; D010166:Palliative Care; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D018570:Risk Assessment; D012307:Risk Factors; D013997:Time Factors; D016896:Treatment Outcome; D014739:Victoria