Factors associated with neurodevelopment for children with single ventricle lesions

Caren S Goldberg, Minmin Lu, Lynn A Sleeper, William T Mahle, J William Gaynor, Ismee A Williams, Kathleen A Mussatto, Richard G Ohye, Eric M Graham, Deborah U Frank, Jeffrey P Jacobs, Catherine Krawczeski, Linda Lambert, Alan Lewis, Victoria L Pemberton, Renee Sananes, Erica Sood, Stephanie B Wechsler, David C Bellinger, Jane W Newburger, 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, Thomas Travison, David F Teitel, Jane W Newburger, Peter Laussen, Pedro del Nido, Roger Breitbart, Jami Levine, Ellen McGrath, Carolyn Dunbar-Masterson, Wyman Lai, Beth Printz, Daphne Hsu, William Hellenbrand, Ismee Williams, Ashwin Prakash, 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, Kathie Hogan, Steven Schwartz, David Nelson, 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 Hawkins, Michael Puchalski, Richard Williams, Linda Lambert, Jun Porter, Marian Shearrow, Brian McCrindle, Joel Kirsh, Chris Caldarone, Elizabeth Radojewski, Svetlana Khaikin, Susan McIntyre, Nancy Slater, Caren S Goldberg, Richard G Ohye, Cheryl Nowak, Nancy Ghanayem, James Tweddell, Kathy Mussatto, Michele 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 Cox, Kirk Kanter, William Mahle, Joel Bond, Leslie French, Jeryl Huckaby, Christian Pizarro, Carol Prospero, Julie Simons, Gina Baffa, 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, Caren S Goldberg, Minmin Lu, Lynn A Sleeper, William T Mahle, J William Gaynor, Ismee A Williams, Kathleen A Mussatto, Richard G Ohye, Eric M Graham, Deborah U Frank, Jeffrey P Jacobs, Catherine Krawczeski, Linda Lambert, Alan Lewis, Victoria L Pemberton, Renee Sananes, Erica Sood, Stephanie B Wechsler, David C Bellinger, Jane W Newburger, 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, Thomas Travison, David F Teitel, Jane W Newburger, Peter Laussen, Pedro del Nido, Roger Breitbart, Jami Levine, Ellen McGrath, Carolyn Dunbar-Masterson, Wyman Lai, Beth Printz, Daphne Hsu, William Hellenbrand, Ismee Williams, Ashwin Prakash, 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, Kathie Hogan, Steven Schwartz, David Nelson, 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 Hawkins, Michael Puchalski, Richard Williams, Linda Lambert, Jun Porter, Marian Shearrow, Brian McCrindle, Joel Kirsh, Chris Caldarone, Elizabeth Radojewski, Svetlana Khaikin, Susan McIntyre, Nancy Slater, Caren S Goldberg, Richard G Ohye, Cheryl Nowak, Nancy Ghanayem, James Tweddell, Kathy Mussatto, Michele 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 Cox, Kirk Kanter, William Mahle, Joel Bond, Leslie French, Jeryl Huckaby, Christian Pizarro, Carol Prospero, Julie Simons, Gina Baffa, 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

Objective: To measure neurodevelopment at 3 years of age in children with single right-ventricle anomalies and to assess its relationship to Norwood shunt type, neurodevelopment at 14 months of age, and patient and medical factors.

Study design: All subjects in the Single Ventricle Reconstruction Trial who were alive without cardiac transplant were eligible for inclusion. The Ages and Stages Questionnaire (ASQ, n = 203) and other measures of behavior and quality of life were completed at age 3 years. Medical history, including measures of growth, feeding, and complications, was assessed through annual review of the records and phone interviews. The Bayley Scales of Infant Development, Second Edition (BSID-II) scores from age 14 months were also evaluated as predictors.

Results: Scores on each ASQ domain were significantly lower than normal (P < .001). ASQ domain scores at 3 years of age varied nonlinearly with 14-month BSID-II. More complications, abnormal growth, and evidence of feeding, vision, or hearing problems were independently associated with lower ASQ scores, although models explained <30% of variation. Type of shunt was not associated with any ASQ domain score or with behavior or quality-of-life measures.

Conclusion: Children with single right-ventricle anomalies have impaired neurodevelopment at 3 years of age. Lower ASQ scores are associated with medical morbidity, and lower BSID-II scores but not with shunt type. Because only a modest percentage of variation in 3-year neurodevelopmental outcome could be predicted from early measures, however, all children with single right-ventricle anomalies should be followed longitudinally to improve recognition of delays.

Trial registration: ClinicalTrials.gov NCT00115934.