Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Randomized Controlled Trial
. 2012 Jul;144(1):152-9.
doi: 10.1016/j.jtcvs.2012.01.016. Epub 2012 Feb 15.

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

James S Tweddell  1 , 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
Collaborators, Affiliations
Randomized Controlled Trial

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

James S Tweddell et al. J Thorac Cardiovasc Surg. 2012 Jul.

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.

PubMed Disclaimer

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.
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

  • A predictive model for neurodevelopmental outcome after the Norwood procedure.
    Mahle WT, Lu M, Ohye RG, William Gaynor J, Goldberg CS, Sleeper LA, Pemberton VL, Mussatto KA, Williams IA, Sood E, Krawczeski CD, Lewis A, Mirarchi N, Scheurer M, Pasquali SK, Pinto N, Jacobs JP, McCrindle BW, Newburger JW. Mahle WT, et al. Pediatr Cardiol. 2013 Feb;34(2):327-33. doi: 10.1007/s00246-012-0450-1. Epub 2012 Aug 3. Pediatr Cardiol. 2013. PMID: 22864647 Free PMC article. Clinical Trial.
  • The Second Interstage Period Is Just as "Risky" as the First in HLHS.
    Ravishankar C. Ravishankar C. JACC Adv. 2024 Apr 10;3(5):100935. doi: 10.1016/j.jacadv.2024.100935. eCollection 2024 May. JACC Adv. 2024. PMID: 38939632 Free PMC article.
  • Technical performance score is associated with outcomes after the Norwood procedure.
    Nathan M, Sleeper LA, Ohye RG, Frommelt PC, Caldarone CA, Tweddell JS, Lu M, Pearson GD, Gaynor JW, Pizarro C, Williams IA, Colan SD, Dunbar-Masterson C, Gruber PJ, Hill K, Hirsch-Romano J, Jacobs JP, Kaltman JR, Kumar SR, Morales D, Bradley SM, Kanter K, Newburger JW; Pediatric Heart Network Investigators. Nathan M, et al. J Thorac Cardiovasc Surg. 2014 Nov;148(5):2208-13, 2214.e1-6. doi: 10.1016/j.jtcvs.2014.05.076. Epub 2014 Jun 19. J Thorac Cardiovasc Surg. 2014. PMID: 25037617 Free PMC article. Clinical Trial.
  • A Shift in Focus.
    Abdulla RI. Abdulla RI. Pediatr Cardiol. 2018 Jun;39(5):857-858. doi: 10.1007/s00246-018-1856-1. Epub 2018 Apr 10. Pediatr Cardiol. 2018. PMID: 29637253 No abstract available.
  • Impact of Initial Shunt Type on Echocardiographic Indices in Children After Single Right Ventricle Palliations.
    Frommelt PC, Hu C, Trachtenberg F, Baffa JM, Boruta RJ, Chowdhury S, Cnota JF, Dragulescu A, Levine JC, Lu J, Mercer-Rosa L, Miller TA, Shah A, Slesnick TC, Stapleton G, Stelter J, Wong P, Newburger JW. Frommelt PC, et al. Circ Cardiovasc Imaging. 2019 Feb;12(2):e007865. doi: 10.1161/CIRCIMAGING.118.007865. Circ Cardiovasc Imaging. 2019. PMID: 30755054 Free PMC article. Clinical Trial.
See all "Cited by" articles

References

    1. Ohye RG, Sleeper LA, Mahony L, Newburger JW, Pearson GD, Lu M, et al. Comparison of shunt types in the Norwood procedure for single-ventricle lesions. N Engl J Med. 2010 May 27;362(21):1980–1992. - PMC - PubMed
    1. Ohye RG, Gaynor JW, Ghanayem NS, Goldberg CS, Laussen PC, Frommelt PC, et al. Design and rationale of a randomized trial comparing the Blalock-Taussig and right ventricle-pulmonary artery shunts in the Norwood procedure. J Thorac Cardiovasc Surg. 2008 Oct;136(4):968–975. - PMC - PubMed
    1. Diez Roux AV, Merkin SS, Arnett D, Chambless L, Massing M, Nieto FJ, et al. Neighborhood of residence and incidence of coronary heart disease. N Engl J Med. 2001 Jul 12;345(2):99–106. - PubMed
    1. Ohye RG, Schonbeck JV, Eghtesady P, Laussen PC, Pizarro C, Shrader P, et al. Cause, timing and location of death in the Single Ventricle Reconstruction Trial. J Thorac Cardiovasc Surg. 20** ;***(*):***-*** [concurrent issue] - PMC - PubMed
    1. Blackstone EH, Naftel DC, Turner ME. The decomposition of time-varying hazard Into phases, each Incorporating a separate stream of concomitant Information. Amer Stat Assoc. 1986;81:615–624.

Publication types

Associated data