Independent Factors Associated With Mortality, Reintervention, and Achievement of Complete Repair in Children With Pulmonary Atresia With Ventricular Septal Defect
Kerstin M. Amark, MD*,
Tara Karamlou, MD ,
Aoife O’Carroll, MS ,
Cathy MacDonald, MD,
Robert M. Freedom, MD,
Shi-Joon Yoo, MD ,
William G. Williams, MD,
Glen S. Van Arsdell, MD,
Christopher A. Caldarone, MD and
Brian W. McCrindle, MD, MPH,*
* Department of Pediatric Cardiology, Göteborg University, The Queen Silvia Children’s Hospital, Göteborg, Sweden
Division of Cardiovascular Surgery, Department of Surgery, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada
Division of Cardiology, Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada
Division of Diagnostic Imaging, Department of Radiology, University of Toronto, The Hospital for Sick Children, Toronto, Ontario, Canada
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Figure 1 Bronchopulmonary arterial anatomy in pulmonary atresia associated with ventricular septal defect (PAVSD) is characterized by a continuous morphologic spectrum, but our definition of major aortopulmonary collateral arteries (MAPCA) patients is supported by the obvious polarization of those in the non-MAPCA group (closed circles) and those in the MAPCA group (open circles). There is a strong negative correlation (r2 = 0.8; p < 0.001) between increasing number of bronchopulmonary segments supplied by the native pulmonary arteries and decreasing number of bronchopulmonary segments with abnormal supply. Abnormal supply = number of dual supplied segments + number of unperfused segments + number of collateral supplied segments; PA = pulmonary artery; RVOT = right ventricular outflow tract.
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Figure 2 Overview of events in all patients admitted with a diagnosis of pulmonary atresia associated with ventricular septal defect (PAVSD). RVOT = right ventricular outflow tract.
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Figure 3 Events in the 171 patients who underwent blinded angiographic review. Patients were classified into two groups, those with major aortopulmonary collateral arteries (n = 53), and those without major aortopulmonary collateral arteries (MAPCAs) (n = 118).
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Figure 4 Proportion of patients reaching definitive repair stratified by era. The percentage of patients undergoing primary complete repair (open bars) increased significantly with increasing era, reflecting an important change in the treatment paradigm for children with pulmonary atresia associated with ventricular septal defect. The number of patients reaching repair after prior palliation (solid bars) also predictably increased with longer duration of follow-up. **p < 0.0001.
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Figure 5 Overall survival from initial operation in 185 children with pulmonary atresia associated with ventricular septal defect. There were 47 deaths after initial operation. The hazard function for death after initial operation was characterized by a steep early phase accounting for 14 events, followed by a more gradual later phase accounting for 33 events. Solid lines represent parametric point estimates enclosed by 70% confidence limits; circles with error bars represent nonparametric estimates, and numbers at bottom represent the number of patients followed up at that point.
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Figure 6 Competing risks depiction of events after initial palliation in 128 children who underwent initial palliation, either right ventricular outflow tract (RVOT) reconstruction (n = 23) or initial systemic-pulmonary artery shunt placement (n = 105). Competing risks analysis predicted that at 10 years after palliation, 68% had achieved complete repair, 22% had died without complete repair, and 10% remained alive without complete repair. Solid lines represent parametric point estimates, dashed lines enclose 70% confidence limits, circles with error bars represent nonparametric estimates, numbers in parentheses indicate the estimated proportion of patients in each state at 10 years from palliation.
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Figure 7 Competing risks diagrams depicting the estimated prevalence of three mutually exclusive end states in 187 patients after initial operation: achievement of complete repair, death without repair, and remaining alive without repair. (A) Decreased rates of transition to complete repair and a higher prevalence of death are seen in a patient with only three bronchopulmonary segments supplied by the true pulmonary arteries and a large number of major aortopulmonary collateral arteries (MAPCAs). (B) Patients with all 18 bronchopulmonary segments supplied by the true pulmonary arteries via the ductus arteriosus, and therefore no MAPCAs, have increased transition rates to complete repair and lower pre-repair attrition.
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Figure 8 Risk-adjusted freedom from reintervention after repair for varying age at complete repair stratified according to major aortopulmonary collateral artery (MAPCA) group. The multivariable equation from the competing risk model was solved twice (for patients with MAPCAs and for those without), each time entering mean values for other predictors. Decreased rates of catheter-based reintervention are illustrated for MAPCA patients when single-stage repair is performed at a later age. Solid lines represent parametric determination of freedom from reintervention for given age at repair; dashed lines enclose 70% confidence intervals.
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