Intermediate-term mortality and cardiac transplantation in infants with single-ventricle lesions: risk factors and their interaction with shunt type

James S Tweddell, Lynn A Sleeper, Richard G Ohye, Ismee A Williams, Lynn Mahony, Christian Pizarro, Victoria L Pemberton, Peter C Frommelt, Scott M Bradley, James F Cnota, Jennifer Hirsch, Paul M Kirshbom, Jennifer S Li, Nancy Pike, Michael Puchalski, Chitra Ravishankar, Jeffrey P Jacobs, Peter C Laussen, Brian W McCrindle, 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 Griffith, William Mahle, Kirk Kanter, 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, James S Tweddell, Lynn A Sleeper, Richard G Ohye, Ismee A Williams, Lynn Mahony, Christian Pizarro, Victoria L Pemberton, Peter C Frommelt, Scott M Bradley, James F Cnota, Jennifer Hirsch, Paul M Kirshbom, Jennifer S Li, Nancy Pike, Michael Puchalski, Chitra Ravishankar, Jeffrey P Jacobs, Peter C Laussen, Brian W McCrindle, 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 Griffith, William Mahle, Kirk Kanter, 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

Objective: The study objective was to identify factors associated with death and cardiac transplantation in infants undergoing the Norwood procedure and to determine differences in associations that might favor the modified Blalock-Taussig shunt or a right ventricle-to-pulmonary artery shunt.

Methods: We used competing risks methodology to analyze death without transplantation, cardiac transplantation, and survival without transplantation. Parametric time-to-event modeling and bootstrapping were used to identify independent predictors.

Results: Data from 549 subjects (follow-up, 2.7 ± 0.9 years) were analyzed. Mortality risk was characterized by early and constant phases; transplant was characterized by only a constant phase. Early phase factors associated with death included lower socioeconomic status (P = .01), obstructed pulmonary venous return (P < .001), smaller ascending aorta (P = .02), and anatomic subtype. Constant phase factors associated with death included genetic syndrome (P < .001) and lower gestational age (P < .001). The right ventricle-to-pulmonary artery shunt demonstrated better survival in the 51% of subjects who were full term with aortic atresia (P < .001). The modified Blalock-Taussig shunt was better among the 4% of subjects who were preterm with a patent aortic valve (P = .003). Lower pre-Norwood right ventricular fractional area change, pre-Norwood surgery, and anatomy other than hypoplastic left heart syndrome were independently associated with transplantation (all P < .03), but shunt type was not (P = .43).

Conclusions: Independent risk factors for intermediate-term mortality include lower socioeconomic status, anatomy, genetic syndrome, and lower gestational age. Term infants with aortic atresia benefited from a right ventricle-to-pulmonary artery shunt, and preterm infants with a patent aortic valve benefited from a modified Blalock-Taussig shunt. Right ventricular function and anatomy, but not shunt type, were associated with transplantation.

Trial registration: ClinicalTrials.gov NCT00115934.

Copyright © 2012 The American Association for Thoracic Surgery. All rights reserved.

Figures

Figure 1
Figure 1
Proportion of subjects in each of three competing mutually exclusive end-states: death, transplant and alive without transplant for all 549 subjects (solid curves). The dashed curves represent the pointwise 68% (1 standard error) confidence bands based on the parametric fit. The squares and associated error bars represent the nonparametric estimates ± one standard error.
Figure 2
Figure 2
A) Parametric survival curve by anatomic subtype of subjects undergoing RVPAS. B) Parametric survival curve by anatomic subtype of subjects undergoing MBTS. C) Parametric survival curve for higher-risk subjects, showing the impact of OPVR on survival among subjects undergoing the Norwood procedure with a MBTS vs. a RVPAS. D) Parametric survival curve for higher-risk subjects, showing the impact of a genetic syndrome on survival among subjects undergoing the Norwood procedure with a MBTS versus a RVPAS. E) Parametric survival curve for lower-risk subjects, showing the impact of shunt type, SES, and aortic diameter on survival of subjects with HLHS-AA/MA. F) Parametric survival curve for lower-risk subjects, showing the impact of shunt type, SES, and aortic diameter on survival of subjects with HLHS-AS/MS.
Figure 2
Figure 2
A) Parametric survival curve by anatomic subtype of subjects undergoing RVPAS. B) Parametric survival curve by anatomic subtype of subjects undergoing MBTS. C) Parametric survival curve for higher-risk subjects, showing the impact of OPVR on survival among subjects undergoing the Norwood procedure with a MBTS vs. a RVPAS. D) Parametric survival curve for higher-risk subjects, showing the impact of a genetic syndrome on survival among subjects undergoing the Norwood procedure with a MBTS versus a RVPAS. E) Parametric survival curve for lower-risk subjects, showing the impact of shunt type, SES, and aortic diameter on survival of subjects with HLHS-AA/MA. F) Parametric survival curve for lower-risk subjects, showing the impact of shunt type, SES, and aortic diameter on survival of subjects with HLHS-AS/MS.
Figure 2
Figure 2
A) Parametric survival curve by anatomic subtype of subjects undergoing RVPAS. B) Parametric survival curve by anatomic subtype of subjects undergoing MBTS. C) Parametric survival curve for higher-risk subjects, showing the impact of OPVR on survival among subjects undergoing the Norwood procedure with a MBTS vs. a RVPAS. D) Parametric survival curve for higher-risk subjects, showing the impact of a genetic syndrome on survival among subjects undergoing the Norwood procedure with a MBTS versus a RVPAS. E) Parametric survival curve for lower-risk subjects, showing the impact of shunt type, SES, and aortic diameter on survival of subjects with HLHS-AA/MA. F) Parametric survival curve for lower-risk subjects, showing the impact of shunt type, SES, and aortic diameter on survival of subjects with HLHS-AS/MS.
Figure 3
Figure 3
Predicted probability of freedom from transplant for subjects with differing cardiac anatomy, RV function, and numbers of pre-Norwood surgeries.

Source: PubMed

3
Iratkozz fel