Early developmental outcome in children with hypoplastic left heart syndrome and related anomalies: the single ventricle reconstruction trial
Jane W Newburger, Lynn A Sleeper, David C Bellinger, Caren S Goldberg, Sarah Tabbutt, Minmin Lu, Kathleen A Mussatto, Ismee A Williams, Kathryn E Gustafson, Seema Mital, Nancy Pike, Erica Sood, William T Mahle, David S Cooper, Carolyn Dunbar-Masterson, Catherine Dent Krawczeski, Alan Lewis, Shaji C Menon, Victoria L Pemberton, Chitra Ravishankar, Teresa W Atz, Richard G Ohye, J William Gaynor, Pediatric Heart Network Investigators, Gail Pearson, Victoria Pemberton, Rae-Ellen Kavey, Mario Stylianou, Marsha Mathis, Lynn Mahony, Lynn Sleeper, Sharon Tennstedt, Steven Colan, Lisa Virzi, Patty Connell, Victoria Muratov, Lisa Wruck, Minmin Lu, Dianne Gallagher, Anne Devine, Julie Schonbeck, Thomas Travison, David F Teitel, Jane W Newburger, Peter Laussen, Pedro del Nido, Roger Breitbart, Jami Levine, Ellen McGrath, Carolyn Dunbar-Masterson, John E Mayer Jr, Frank Pigula, Emile A Bacha, Francis Fynn-Thompson, Wyman Lai, Beth Printz, Daphne Hsu, William Hellenbrand, Ismee Williams, Ashwin Prakash, Seema Mital, Ralph Mosca, Darlene Servedio, Rozelle Corda, Rosalind Korsin, Mary Nash, Victoria L Vetter, Sarah Tabbutt, J William Gaynor, Chitra Ravishankar, Thomas Spray, Meryl Cohen, Marisa Nolan, Stephanie Piacentino, Sandra DiLullo, Nicole Mirarchi, D Woodrow Benson, Catherine Dent Krawczeski, Lois Bogenschutz, Teresa Barnard, Michelle Hamstra, Rachel Griffiths, Kathryn Hogan, Steven Schwartz, David Nelson, Pirooz Eghtesady, Page A W Anderson, Jennifer Li, Wesley Covitz, Kari Crawford, Michael Hines, James Jaggers, Theodore Koutlas, Charlie Sang Jr, Lori Jo Sutton, Mingfen Xu, J Philip Saul, Andrew Atz, Girish Shirali, Scott Bradley, Eric Graham, Teresa Atz, Patricia Infinger, L LuAnn Minich, John A Hawkins, Michael Puchalski, Richard V Williams, Peter C Kouretas, Linda M Lambert, Marian E Shearrow, Jun A Porter, Brian McCrindle, Joel Kirsh, Chris Caldarone, Elizabeth Radojewski, Svetlana Khaikin, Susan McIntyre, Nancy Slater, Caren S Goldberg, Richard G Ohye, Cheryl Nowak, Nancy S Ghanayem, James S Tweddell, Kathleen A Mussatto, Michele A Frommelt, Peter C Frommelt, Lisa Young-Borkowski, Alan Lewis, Vaughn Starnes, Nancy Pike, Jeffrey P Jacobs, James A Quintessenza, Paul J Chai, David S Cooper, J Blaine John, James C Huhta, Tina Merola, Tracey Grifith, Kirk Kanter, William Mahle, Joel Bond, Jeryl Huckaby, Christian Pizarro, Carol Prospero, Julie Simons, Gina Baffa, Wolfgang A Radtke, Ilana Zeltzer, Tia Tortoriello, Deborah McElroy, Deborah Town, John Rhodes, J Curt Fudge, Peter Frommelt, Gerald Marx, Catherine Stolle, Michael Artman, Erle Austin, Timothy Feltes, Julie Johnson, Thomas Klitzner, Jeffrey Krischer, G Paul Matherne, John Kugler, Rae-Ellen Kavey, David J Driscoll, Mark Galantowicz, Sally A Hunsberger, Thomas J Knight, Holly Taylor, Catherine L Webb, Jane W Newburger, Lynn A Sleeper, David C Bellinger, Caren S Goldberg, Sarah Tabbutt, Minmin Lu, Kathleen A Mussatto, Ismee A Williams, Kathryn E Gustafson, Seema Mital, Nancy Pike, Erica Sood, William T Mahle, David S Cooper, Carolyn Dunbar-Masterson, Catherine Dent Krawczeski, Alan Lewis, Shaji C Menon, Victoria L Pemberton, Chitra Ravishankar, Teresa W Atz, Richard G Ohye, J William Gaynor, Pediatric Heart Network Investigators, Gail Pearson, Victoria Pemberton, Rae-Ellen Kavey, Mario Stylianou, Marsha Mathis, Lynn Mahony, Lynn Sleeper, Sharon Tennstedt, Steven Colan, Lisa Virzi, Patty Connell, Victoria Muratov, Lisa Wruck, Minmin Lu, Dianne Gallagher, Anne Devine, Julie Schonbeck, Thomas Travison, David F Teitel, Jane W Newburger, Peter Laussen, Pedro del Nido, Roger Breitbart, Jami Levine, Ellen McGrath, Carolyn Dunbar-Masterson, John E Mayer Jr, Frank Pigula, Emile A Bacha, Francis Fynn-Thompson, Wyman Lai, Beth Printz, Daphne Hsu, William Hellenbrand, Ismee Williams, Ashwin Prakash, Seema Mital, Ralph Mosca, Darlene Servedio, Rozelle Corda, Rosalind Korsin, Mary Nash, Victoria L Vetter, Sarah Tabbutt, J William Gaynor, Chitra Ravishankar, Thomas Spray, Meryl Cohen, Marisa Nolan, Stephanie Piacentino, Sandra DiLullo, Nicole Mirarchi, D Woodrow Benson, Catherine Dent Krawczeski, Lois Bogenschutz, Teresa Barnard, Michelle Hamstra, Rachel Griffiths, Kathryn Hogan, Steven Schwartz, David Nelson, Pirooz Eghtesady, Page A W Anderson, Jennifer Li, Wesley Covitz, Kari Crawford, Michael Hines, James Jaggers, Theodore Koutlas, Charlie Sang Jr, Lori Jo Sutton, Mingfen Xu, J Philip Saul, Andrew Atz, Girish Shirali, Scott Bradley, Eric Graham, Teresa Atz, Patricia Infinger, L LuAnn Minich, John A Hawkins, Michael Puchalski, Richard V Williams, Peter C Kouretas, Linda M Lambert, Marian E Shearrow, Jun A Porter, Brian McCrindle, Joel Kirsh, Chris Caldarone, Elizabeth Radojewski, Svetlana Khaikin, Susan McIntyre, Nancy Slater, Caren S Goldberg, Richard G Ohye, Cheryl Nowak, Nancy S Ghanayem, James S Tweddell, Kathleen A Mussatto, Michele A Frommelt, Peter C Frommelt, Lisa Young-Borkowski, Alan Lewis, Vaughn Starnes, Nancy Pike, Jeffrey P Jacobs, James A Quintessenza, Paul J Chai, David S Cooper, J Blaine John, James C Huhta, Tina Merola, Tracey Grifith, Kirk Kanter, William Mahle, Joel Bond, Jeryl Huckaby, Christian Pizarro, Carol Prospero, Julie Simons, Gina Baffa, Wolfgang A Radtke, Ilana Zeltzer, Tia Tortoriello, Deborah McElroy, Deborah Town, John Rhodes, J Curt Fudge, Peter Frommelt, Gerald Marx, Catherine Stolle, Michael Artman, Erle Austin, Timothy Feltes, Julie Johnson, Thomas Klitzner, Jeffrey Krischer, G Paul Matherne, John Kugler, Rae-Ellen Kavey, David J Driscoll, Mark Galantowicz, Sally A Hunsberger, Thomas J Knight, Holly Taylor, Catherine L Webb
Abstract
Background: Survivors of the Norwood procedure may experience neurodevelopmental impairment. Clinical trials to improve outcomes have focused primarily on methods of vital organ support during cardiopulmonary bypass.
Methods and results: In the Single Ventricle Reconstruction trial of the Norwood procedure with modified Blalock-Taussig shunt versus right-ventricle-to-pulmonary-artery shunt, 14-month neurodevelopmental outcome was assessed by use of the Psychomotor Development Index (PDI) and Mental Development Index (MDI) of the Bayley Scales of Infant Development-II. We used multivariable regression to identify risk factors for adverse outcome. Among 373 transplant-free survivors, 321 (86%) returned at age 14.3 ± 1.1 (mean ± SD) months. Mean PDI (74 ± 19) and MDI (89 ± 18) scores were lower than normative means (each P<0.001). Neither PDI nor MDI score was associated with type of Norwood shunt. Independent predictors of lower PDI score (R(2)=26%) were clinical center (P=0.003), birth weight <2.5 kg (P=0.023), longer Norwood hospitalization (P<0.001), and more complications between Norwood procedure discharge and age 12 months (P<0.001). Independent risk factors for lower MDI score (R(2)=34%) included center (P<0.001), birth weight <2.5 kg (P=0.04), genetic syndrome/anomalies (P=0.04), lower maternal education (P=0.04), longer mechanical ventilation after the Norwood procedure (P<0.001), and more complications after Norwood discharge to age 12 months (P<0.001). We found no significant relationship of PDI or MDI score to perfusion type, other aspects of vital organ support (eg, hematocrit, pH strategy), or cardiac anatomy.
Conclusions: Neurodevelopmental impairment in Norwood survivors is more highly associated with innate patient factors and overall morbidity in the first year than with intraoperative management strategies. Improved outcomes are likely to require interventions that occur outside the operating room.
Clinical trial registration: URL: http://www.clinicaltrials.gov. Unique identifier: NCT00115934.
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Source: PubMed