James M Meza1, Edward Hickey1, Brian McCrindle2, Eugene Blackstone3, Brett Anderson4, David Overman5, James K Kirklin6, Tara Karamlou7, Christopher Caldarone1, Richard Kim8, William DeCampli9, Marshall Jacobs10, Kristine Guleserian11, Jeffrey P Jacobs12, Robert Jaquiss13. 1. Division of Cardiovascular Surgery, The Hospital for Sick Children, Toronto, Ontario. 2. Division of Pediatric Cardiology, The Hospital for Sick Children, Toronto, Ontario. 3. Division of Thoracic and Cardiovascular Surgery and Quantitative Health Sciences, Cleveland Clinic, Cleveland, Ohio. 4. Division of Cardiology, Morgan-Stanley Children's Hospital/New York Presbyterian Hospital, New York, New York. 5. Division of Pediatric Cardiovascular Surgery, Children's Hospitals and Clinics of Minnesota, Minneapolis, Minnesota. 6. Division of Cardiothoracic Surgery, University of Alabama at Birmingham, Birmingham, Alabama. 7. Division of Thoracic and Cardiovascular Surgery, Phoenix Children's Hospital, Phoenix, Arizona. 8. Division of Cardiothoracic Surgery, Los Angeles Children's Hospital, Los Angeles, California. 9. Division of Pediatric Cardiac Surgery, Arnold Palmer Hospital for Children, Orlando, Florida. 10. Division of Cardiac Surgery, Johns Hopkins Heart and Vascular Institute, Baltimore, Maryland. 11. Division of Cardiovascular Surgery, Niklaus Children's Hospital, Miami, Florida. 12. Division of Cardiovascular Surgery, Johns Hopkins All Children's Hospital, St. Petersburg, Florida. 13. Division of Pediatric Cardiothoracic Surgery, Children's Medical Center, Dallas, Texas. Electronic address: robert.jaquiss@utsouthwestern.edu.
Abstract
BACKGROUND: The effect of the timing of stage-2-palliation (S2P) on survival through single ventricle palliation remains unknown. This study investigated the optimal timing of S2P that minimizes pre-S2P attrition and maximizes post-S2P survival. METHODS: The Congenital Heart Surgeons' Society's critical left ventricular outflow tract obstruction cohort was used. Survival analysis was performed using multiphase parametric hazard analysis. Separate risk factors for death after the Norwood and after S2P were identified. Based on the multivariable models, infants were stratified as low, intermediate, or high risk. Cumulative 2-year, post-Norwood survival was predicted. Optimal timing was determined using conditional survival analysis and plotted as 2-year, post-Norwood survival versus age at S2P. RESULTS: A Norwood operation was performed in 534 neonates from 21 institutions. The S2P was performed in 71%, at a median age of 5.1 months (IQR: 4.3 to 6.0), and 22% died after Norwood. By 5 years after S2P, 10% of infants had died. For low- and intermediate-risk infants, performing S2P after age 3 months was associated with 89% ± 3% and 82% ± 3% 2-year survival, respectively. Undergoing an interval cardiac reoperation or moderate-severe right ventricular dysfunction before S2P were high-risk features. Among high-risk infants, 2-year survival was 63% ± 5%, and even lower when S2P was performed before age 6 months. CONCLUSIONS: Performing S2P after age 3 months may optimize survival of low- and intermediate-risk infants. High-risk infants are unlikely to complete three-stage palliation, and early S2P may increase their risk of mortality. We infer that early referral for cardiac transplantation may increase their chance of survival.
BACKGROUND: The effect of the timing of stage-2-palliation (S2P) on survival through single ventricle palliation remains unknown. This study investigated the optimal timing of S2P that minimizes pre-S2P attrition and maximizes post-S2P survival. METHODS: The Congenital Heart Surgeons' Society's critical left ventricular outflow tract obstruction cohort was used. Survival analysis was performed using multiphase parametric hazard analysis. Separate risk factors for death after the Norwood and after S2P were identified. Based on the multivariable models, infants were stratified as low, intermediate, or high risk. Cumulative 2-year, post-Norwood survival was predicted. Optimal timing was determined using conditional survival analysis and plotted as 2-year, post-Norwood survival versus age at S2P. RESULTS: A Norwood operation was performed in 534 neonates from 21 institutions. The S2P was performed in 71%, at a median age of 5.1 months (IQR: 4.3 to 6.0), and 22% died after Norwood. By 5 years after S2P, 10% of infants had died. For low- and intermediate-risk infants, performing S2P after age 3 months was associated with 89% ± 3% and 82% ± 3% 2-year survival, respectively. Undergoing an interval cardiac reoperation or moderate-severe right ventricular dysfunction before S2P were high-risk features. Among high-risk infants, 2-year survival was 63% ± 5%, and even lower when S2P was performed before age 6 months. CONCLUSIONS: Performing S2P after age 3 months may optimize survival of low- and intermediate-risk infants. High-risk infants are unlikely to complete three-stage palliation, and early S2P may increase their risk of mortality. We infer that early referral for cardiac transplantation may increase their chance of survival.
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