Prenatal diagnosis and risk factors for preoperative death in neonates with single right ventricle and systemic outflow obstruction: screening data from the Pediatric Heart Network Single Ventricle Reconstruction Trial(∗)

Andrew M Atz, Thomas G Travison, Ismee A Williams, Gail D Pearson, Peter C Laussen, William T Mahle, Amanda L Cook, Joel A Kirsh, Mark Sklansky, Svetlana Khaikin, Caren Goldberg, Michele Frommelt, Catherine Krawczeski, Michael D Puchalski, Jeffrey P Jacobs, Jeanne M Baffa, Jack Rychik, Richard G Ohye, Pediatric Heart Network Investigators, Gail Pearson, Victoria Pemberton, Rae-Ellen Kavey, Mario Stylianou, Marsha Mathis, 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 Wisconsin, 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, Blaine J 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, Andrew M Atz, Thomas G Travison, Ismee A Williams, Gail D Pearson, Peter C Laussen, William T Mahle, Amanda L Cook, Joel A Kirsh, Mark Sklansky, Svetlana Khaikin, Caren Goldberg, Michele Frommelt, Catherine Krawczeski, Michael D Puchalski, Jeffrey P Jacobs, Jeanne M Baffa, Jack Rychik, Richard G Ohye, Pediatric Heart Network Investigators, Gail Pearson, Victoria Pemberton, Rae-Ellen Kavey, Mario Stylianou, Marsha Mathis, 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 Wisconsin, 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, Blaine J 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

Objectives: The purpose of this analysis was to assess preoperative risk factors before the first-stage Norwood procedure in infants with hypoplastic left heart syndrome and related single-ventricle lesions and to evaluate practice patterns in prenatal diagnosis, as well as the role of prenatal diagnosis in outcome.

Methods: Data from all live births with morphologic single right ventricle and systemic outflow obstruction screened for the Pediatric Heart Network's Single Ventricle Reconstruction Trial were used to investigate prenatal diagnosis and preoperative risk factors. Demographics, gestational age, prenatal diagnosis status, presence of major extracardiac congenital abnormalities, and preoperative mortality rates were recorded.

Results: Of 906 infants, 677 (75%) had prenatal diagnosis, 15% were preterm (<37 weeks' gestation), and 16% were low birth weight (<2500 g). Rates of prenatal diagnosis varied by study site (59% to 85%, P < .0001). Major extracardiac congenital abnormalities were less prevalent in those born after prenatal diagnosis (6% vs 10%, P = .03). There were 26 (3%) deaths before Norwood palliation; preoperative mortality did not differ by prenatal diagnosis status (P = .49). In multiple logistic regression models, preterm birth (P = .02), major extracardiac congenital abnormalities (P < .0001), and obstructed pulmonary venous return (P = .02) were independently associated with preoperative mortality.

Conclusions: Prenatal diagnosis occurred in 75%. Preoperative death was independently associated with preterm birth, obstructed pulmonary venous return, and major extracardiac congenital abnormalities. Adjusted for gestational age and the presence of obstructed pulmonary venous return, the estimated odds of preoperative mortality were 10 times greater for subjects with a major extracardiac congenital abnormality.

Copyright © 2010 The American Association for Thoracic Surgery. All rights reserved.

Figures

Figure 1
Figure 1
Kernel density estimates of gestational age according to prenatal diagnosis status.
Figure 2
Figure 2
Center-specific proportions of subjects with prenatal diagnosis, versus number of subjects screened (N = 906). The 15 trial centers are displayed as circular symbols sized in relative proportion to the square root of their respective sample sizes.

Source: PubMed

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