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Pediatric Cardiology

, Volume 39, Issue 5, pp 955–966 | Cite as

Survival to Stage II with Ventricular Dysfunction: Secondary Analysis of the Single Ventricle Reconstruction Trial

  • Emilie Jean-St-Michel
  • James M. Meza
  • Jonathon Maguire
  • John Coles
  • Brian W. McCrindle
Original Article
  • 242 Downloads

Abstract

Ventricular dysfunction affects survival in patients with single right ventricle (RV), and remains one of the primary indications for heart transplantation. Since it is challenging to predict the capacity of patients with ventricular dysfunction to proceed to the stage II procedure, we sought to identify factors that would be associated with death or heart transplantation without achieving stage II for single RV patients with ventricular dysfunction after Norwood procedure. The Single Ventricle Reconstruction (SVR) trial public-use database was used. Patients with a RV ejection fraction less than 44% or a RV fractional area of change less than 35% on the post-Norwood echocardiogram were included. Parametric risk hazard analysis was used to identify risk factors for death or transplantation without achieving stage II. Of 365 patients with ventricular function measurements on the post-Norwood echocardiogram, 123 (34%) patients had RV dysfunction. The transplantation-free survival was significantly lower for those with ventricular dysfunction compared to those with normal function (log rank Chi-square = 4.23, p = 0.04). Furthermore, having a Blalock–Taussig (BT) shunt, a large RV, a post-Norwood infectious complication, and a surgeon who performs five or less Norwood per year were independent risk factors for death or transplantation without achieving stage II. The predicted 6-month transplantation-free survival for patients with all four identified risk factors was 1% (70% CI 0–13%). Early heart transplantation referral might be considered for post-Norwood patients with BT shunt and RV dysfunction, especially if other high-risk features are present.

Keywords

Hypoplastic left heart syndrome Norwood Transplantation Parametric Blalock–Taussig shunt 

Notes

Compliance with Ethical Standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical Approval

This article does not contain any studies with human participants performed by any of the authors since we used the dataset provided by the Pediatric Heart Network which has been completely de-identified, and is now publically accessible via the Pediatric Heart Network website.

Supplementary material

246_2018_1845_MOESM1_ESM.tiff (109 kb)
Supplement Fig. 1 Distribution of time in days from the Norwood procedure to the post-Norwood echocardiogram in days. This graph represents the number of days between the Norwood procedure and the post-Norwood echocardiogram for each subject within the ventricular dysfunction cohort (TIFF 108 KB)
246_2018_1845_MOESM2_ESM.tiff (22 kb)
Supplement Fig. 2 Time to death, heart transplant, or stage II. The time to death, heart transplant or stage II was defined as the time from the Norwood procedure to the post-Norwood echocardiogram plus the time from post-Norwood echocardiogram to the event. This graph represent the observation period included in our analysis after adjusting for left censoring (TIFF 22 KB)
246_2018_1845_MOESM3_ESM.docx (33 kb)
Supplementary material 3 (DOCX 33 KB)

References

  1. 1.
    Frommelt PC, Guey LT, Minich LL, Bhat M, Bradley TJ, Colan SD, Ensing G, Gorentz J, Heydarian H, John JB, Lai WW, Levine JC, Mahle WT, Miller SG, Ohye RG, Pearson GD, Shirali GS, Wong PC, Cohen MS, for the Pediatric Heart Network Investigators (2012) Does initial shunt type for the norwood procedure affect echocardiographic measures of cardiac size and function during infancy?: the single ventricle reconstruction trial. Circulation 125:2630–2638.  https://doi.org/10.1161/CIRCULATIONAHA.111.072694 CrossRefPubMedPubMedCentralGoogle Scholar
  2. 2.
    Wilder TJ, McCrindle BW, Phillips AB, Blackstone EH, Rajeswaran J, Williams WG, DeCampli WM, Jacobs JP, Jacobs ML, Karamlou T, Kirshbom PM, Lofland GK, Ziemer G, Hickey EJ (2015) Survival and right ventricular performance for matched children after stage-1 Norwood: modified Blalock-Taussig shunt versus right-ventricle-to-pulmonary-artery conduit. J Thorac Cardiovasc Surg 150:1440–1452.  https://doi.org/10.1016/J.JTCVS.2015.06.069 CrossRefPubMedGoogle Scholar
  3. 3.
    Jean-St-Michel E, Chetan D, Schwartz SM, Van Arsdell GS, Floh AA, Honjo O, Conway J (2016) Outcomes in patients with persistent ventricular dysfunction after stage I palliation for hypoplastic left heart syndrome. Pediatr Cardiol 37:239–247.  https://doi.org/10.1007/s00246-015-1268-4 CrossRefPubMedGoogle Scholar
  4. 4.
    Chetan D, Kotani Y, Jacques F, Poynter JA, Benson LN, Lee K-J, Chaturvedi RR, Friedberg MK, Van Arsdell GS, Caldarone CA, Honjo O (2013) Surgical palliation strategy does not affect interstage ventricular dysfunction or atrioventricular valve regurgitation in children with hypoplastic left heart syndrome and variants. Circulation 128:S205–S212.  https://doi.org/10.1161/CIRCULATIONAHA.112.000380 CrossRefPubMedGoogle Scholar
  5. 5.
    Tabbutt S, Ghanayem N, Ravishankar C, Sleeper LA, Cooper DS, Frank DU, Lu M, Pizarro C, Frommelt P, Goldberg CS, Graham EM, Krawczeski CD, Lai WW, Lewis A, Kirsh JA, Mahony L, Ohye RG, Simsic J, Lodge AJ, Spurrier E, Stylianou M, Laussen P (2012) Risk factors for hospital morbidity and mortality after the Norwood procedure: a report from the pediatric heart network single ventricle reconstruction trial. J Thorac Cardiovasc Surg 144:882–895.  https://doi.org/10.1016/j.jtcvs.2012.05.019 CrossRefPubMedPubMedCentralGoogle Scholar
  6. 6.
    Ghanayem NS, Allen KR, Tabbutt S, Atz AM, Clabby ML, Cooper DS, Eghtesady P, Frommelt PC, Gruber PJ, Hill KD, Kaltman JR, Laussen PC, Lewis AB, Lurito KJ, Minich LL, Ohye RG, Schonbeck JV, Schwartz SM, Singh RK, Goldberg CS, Pediatric Heart Network Investigators (2012) Interstage mortality after the Norwood procedure: results of the multicenter single ventricle reconstruction trial. J Thorac Cardiovasc Surg 144:896–906.  https://doi.org/10.1016/j.jtcvs.2012.05.020 CrossRefPubMedPubMedCentralGoogle Scholar
  7. 7.
    Newburger JW, Sleeper LA, Frommelt PC, Pearson GD, Mahle WT, Chen S, Dunbar-Masterson C, Mital S, Williams IA, Ghanayem NS, Goldberg CS, Jacobs JP, Krawczeski CD, Lewis AB, Pasquali SK, Pizarro C, Gruber PJ, Atz AM, Khaikin S, Gaynor JW, Ohye RG, Pediatric Heart Network Investigators (2014) Transplantation-free survival and interventions at 3 years in the single ventricle reconstruction trial. Circulation 129:2013–2020.  https://doi.org/10.1161/CIRCULATIONAHA.113.006191 CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    Muthurangu V, Simpson JM, Razavi RS (2005) Spontaneous improvement of severe right ventricular dysfunction in the setting of hypoplasia of the left heart. Cardiol Young 15:75–78.  https://doi.org/10.1017/S1047951105000156 CrossRefPubMedGoogle Scholar
  9. 9.
    O’Connor MJ, Elias MD, Cohen MS, Quartermain MD (2012) Outcomes of infants undergoing superior cavopulmonary connection in the presence of ventricular dysfunction. Pediatr Cardiol 33:547–553.  https://doi.org/10.1007/s00246-011-0147 CrossRefPubMedGoogle Scholar
  10. 10.
    Kulkarni A, Neugebauer R, Lo Y, Gao Q, Lamour JM, Weinstein S, Hsu DT (2016) Outcomes and risk factors for listing for heart transplantation after the Norwood procedure: an analysis of the single ventricle reconstruction trial. J Heart Lung Transplant 35:306–311.  https://doi.org/10.1016/j.healun.2015.10.033 CrossRefPubMedGoogle Scholar
  11. 11.
    Everitt MD, Boyle GJ, Schechtman KB, Zheng J, Bullock EA, Kaza AK, Dipchand AI, Naftel DC, Kirklin JK, Canter CE (2012) Early survival after heart transplant in young infants is lowest after failed single-ventricle palliation: a multi-institutional study. J Heart Lung Transplant 31:509–516.  https://doi.org/10.1016/j.healun.2011.12.013 CrossRefPubMedGoogle Scholar
  12. 12.
    Ohye RG, Sleeper LA, Mahony L, Newburger JW, Pearson GD, Lu M, Goldberg CS, Tabbutt S, Frommelt PC, Ghanayem NS, Laussen PC, Rhodes JF, Lewis AB, Mital S, Ravishankar C, Williams IA, Dunbar-Masterson C, Atz AM, Colan S, Minich LL, Pizarro C, Kanter KR, Jaggers J, Jacobs JP, Krawczeski CD, Pike N, McCrindle BW, Virzi L, Gaynor JW (2010) Comparison of shunt types in the Norwood procedure for single-ventricle lesions. N Engl J Med 362:1980–1992.  https://doi.org/10.1056/NEJMoa0912461 CrossRefPubMedPubMedCentralGoogle Scholar
  13. 13.
    Ohye RG, Gaynor JW, Ghanayem NS, Goldberg CS, Laussen PC, Frommelt PC, Newburger JW, Pearson GD, Tabbutt S, Wernovsky G, Wruck LM, Atz AM, Colan SD, Jaggers J, McCrindle BW, Prakash A, Puchalski MD, Sleeper LA, Stylianou MP, Mahony L (2008) 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 136:968–975.  https://doi.org/10.1016/j.jtcvs.2008.01.013 CrossRefPubMedPubMedCentralGoogle Scholar
  14. 14.
    Pavlicek M, Wahl A, Rutz T, de Marchi SF, Hille R, Wustmann K, Steck H, Eigenmann C, Schwerzmann M, Seiler C (2011) Right ventricular systolic function assessment: rank of echocardiographic methods vs. cardiac magnetic resonance imaging. Eur J Echocardiogr 12:871–880.  https://doi.org/10.1093/ejechocard/jer CrossRefPubMedGoogle Scholar
  15. 15.
    Ling LF, Marwick TH (2012) Echocardiographic assessment of right ventricular function. JCMG 5:747–753.  https://doi.org/10.1016/j.jcmq.2011.08.026 CrossRefGoogle Scholar
  16. 16.
    Rudski LG, Lai WW, Afilalo J, Hua L, Chandrasekaran K, Solomon SD, Louie EK, Schiller NB (2010) Guidelines for the echocardiographic assessment of the right heart in adults: a report from the american society of echocardiography. J Am Soc Echocardiogr 23:685–713.  https://doi.org/10.1016/j.echo.2010.05.010 CrossRefPubMedGoogle Scholar
  17. 17.
    Blackstone EH, Naftel DC, Turner JRME. (1986) The decomposition of time-varying hazard into phases, each incorporating a separate stream of concomitant information. J Am Stat Assoc 81:615–624CrossRefGoogle Scholar
  18. 18.
    Tweddell JS, Sleeper LA, Ohye RG, Williams IA, Mahony L, Pizarro C, Pemberton VL, Frommelt PC, Bradley SM, Cnota JF, Hirsch J, Kirshbom PM, Li JS, Pike N, Puchalski M, Ravishankar C, Jacobs JP, Laussen PC, McCrindle BW (2012) Intermediate-term mortality and cardiac transplantation in infants with single-ventricle lesions: Risk factors and their interaction with shunt type. J Thorac Cardiovasc Surg 144:152–159.  https://doi.org/10.1016/j.jtcvs.2012.01.016 CrossRefPubMedPubMedCentralGoogle Scholar
  19. 19.
    Rubin DB (2004) Multiple imputation for nonresponse in surveys. Wiley, HobokenGoogle Scholar
  20. 20.
    Austin PC, Tu JV (2004) Bootstrap methods for developing predictive models. Am Stat 58:131–137CrossRefGoogle Scholar
  21. 21.
    Carlo WF, West SC, McCulloch M, Naftel DC, Pruitt E, Kirklin JK, Hubbard M, Molina KM, Gajarski R (2016) Impact of initial Norwood shunt type on young hypoplastic left heart syndrome patients listed for heart transplant: a multi-institutional study. J Heart Lung Transplant 35:301–305.  https://doi.org/10.1016/j.healun.2015.10.032 CrossRefPubMedGoogle Scholar
  22. 22.
    Schwartz SM, Lu M, Ohye RG, Hill KD, Atz AM, Naim MY, Williams IA, Goldberg CS, Lewis A, Pigula F, Manning P, Pizarro C, Chai P, McCandless R, Dunbar-Masterson C, Kaltman JR, Kanter K, Sleeper LA, Schonbeck JV, Ghanayem N, Pediatric Heart Network Investigators (2014) Risk factors for prolonged length of stay after the stage 2 procedure in the single-ventricle reconstruction trial. J Thorac Cardiovasc Surg 147:1791–1798.  https://doi.org/10.1016/j.jtcvs.2013.07.063 CrossRefPubMedGoogle Scholar
  23. 23.
    Bradley SM, Simsic JM, McQuinn TC, Habib DM, Shirali GS, Atz AM (2004) Hemodynamic status after the Norwood procedure: a comparison of right ventricle-to-pulmonary artery connection versus modified Blalock-Taussig shunt. Ann Thorac Surg 78:933–941.  https://doi.org/10.1016/j.athoracsur.2004.04.016 CrossRefPubMedGoogle Scholar
  24. 24.
    Ghanayem NS, Jaquiss RDB, Cava JR, Frommelt PC, Mussatto KA, Hoffman GM, Tweddell JS (2006) Right ventricle-to-pulmonary artery conduit versus Blalock-Taussig shunt: a hemodynamic comparison. Ann Thorac Surg 82:1603–1610.  https://doi.org/10.1016/j.athoracsur.2006.05.103 CrossRefPubMedGoogle Scholar
  25. 25.
    DeCampli WM, Tsai FW, Argueta-Morales IR, Smith C, Munro HM (2013) The effect of epinephrine on coronary flow in the setting of a systemic-to-pulmonary artery shunt. World J Pediatr Congenit Heart Surg 4:373–379.  https://doi.org/10.1177/2150135113490760 CrossRefPubMedGoogle Scholar
  26. 26.
    Driscol TE, Moir TW, Ecksteine RW (1964) Autoregulation of coronary blood flow: effect of interarterial pressure gradients. Circ Res 15:103–111CrossRefPubMedGoogle Scholar
  27. 27.
    Donnelly JP, Raffel DM, Shulkin BL, Corbett JR, Bove EL, Mosca RS, Kulik TJ (1998) Resting coronary flow and coronary flow reserve in human infants after repair or palliation of congenital heart defects as measured by positron emission tomography. J Thorac Cardiovasc Surg 115:103–110CrossRefPubMedGoogle Scholar
  28. 28.
    Baird RJ, Adiseshiah M (1976) The response of diastolic myocardial tissue pressure and regional coronary blood flow to increased preload from blood, colloid, crystalloid. Surgery 79:644–651PubMedGoogle Scholar
  29. 29.
    Honjo O, Atlin CR, Mertens L, Al-Radi OO, Redington AN, Caldarone CA, VanArsdell GS (2011) Atrioventricular valve repair in patients with functional single-ventricle physiology: impact of ventricular and valve function and morphology on survival and reintervention. J Thorac Cardiovasc Surg 142(2):326–335.  https://doi.org/10.1016/j.jtcvs.2010.11.060b CrossRefPubMedGoogle Scholar
  30. 30.
    Hsu DT, Zak V, Mahony L, Sleeper LA, Sleeper LA, Atz AM, Levine JC, Barker PC, Ravishankar C, McCrindle BW, Williams RV, Altmann K, Ghanayem NS, Margossian R, Chung WK, Border WL, Pearson GD, Stylianou MP, Mital S, for the Pediatric Heart Network Investigators (2010) Enalapril in infants with single ventricle: results of a Multicenter Randomized Trial. Circulation 122:333–340.  https://doi.org/10.1161/CIRCULATIONAHA.109.927988 CrossRefPubMedPubMedCentralGoogle Scholar
  31. 31.
    Shaddy RE, Boucek MM, Hsu DT, Boucek RJ, Canter CE, Mahony L, Ross RD, Pahl E, Blume ED, Dodd DA, Rosenthal DN, Burr J, LaSalle B, Holubkov R, Lukas MA, Tani LY, Pediatric Carvedilol Study Group (2007) Carvedilol for children and adolescents with heart failure: a randomized controlled trial. JAMA 298:1171–1179.  https://doi.org/10.1001/jama.298.10.1171 CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Division of Cardiology, The Labatt Family Heart Centre, Department of Pediatrics, The Hospital for Sick ChildrenUniversity of TorontoTorontoCanada
  2. 2.Division of Cardiovascular Surgery, The Labatt Family Heart Centre, Department of Surgery, The Hospital for Sick ChildrenUniversity of TorontoTorontoCanada
  3. 3.Li Ka Shing Knowledge Institute of St. Michael’s hospital, Department of Pediatrics, St. Michael’s HospitalUniversity of TorontoTorontoCanada

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