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Published on: February 11, 2022
Survival into adulthood of patients with atrial isomerism undergoing cardiac surgery
Jayapadman Bhaskar1, John C Galati2, Paul Brooks3
1Department of Cardiac Surgery, Royal Children's Hospital, Melbourne, Australia.
Insights
Patients with atrial isomerism face poor survival, with obstructed pulmonary venous drainage and asplenia being key mortality predictors. Survival rates remain low, especially for those with right atrial isomerism, indicating a need for improved management strategies.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Medical Genetics
Background:
- Atrial isomerism encompasses a spectrum of complex congenital anomalies.
- Understanding factors influencing outcomes is crucial for clinical management.
Purpose of the Study:
- To identify determinants of adverse outcomes in patients with atrial isomerism.
- To analyze survival predictors for left and right atrial isomerism separately.
Main Methods:
- Retrospective analysis of patients with left and right atrial isomerism.
- Survival determinants were assessed for the entire cohort and subgroups.
- Statistical analysis included hazard ratios and confidence intervals.
Main Results:
- Overall 25-year survival was 62%.
- Independent mortality predictors included obstructed total anomalous pulmonary venous drainage and asplenia.
- Left atrial isomerism demonstrated better survival than right atrial isomerism.
- Specific predictors varied between left (dextrocardia, pulmonary stenosis) and right (biventricular repair, obstructed TAPVD) isomerism.
Conclusions:
- A significant number of patients with atrial isomerism do not survive to adulthood.
- Obstructed pulmonary venous drainage is associated with very poor survival.
- Biventricular repair does not improve survival in right atrial isomerism.
Objectives:
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.
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