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Pediatric Cardiology

, Volume 39, Issue 8, pp 1514–1522 | Cite as

Systemic Ventricular Dysfunction Between Stage One and Stage Two Palliation

  • Thomas J. Kulik
  • Lynn A. Sleeper
  • Christina VanderPluym
  • Stephen P. Sanders
Original Article

Abstract

Infants with a single ventricle can develop systemic ventricular dysfunction (SVD) after stage 1 operation, but available information is sparse. We reviewed our patients having Norwood, Sano, or hybrid procedures to better understand this problem. We conducted a retrospective, case-controlled cohort study of 267 patients having stage1 operation, examining outcomes between stages 1 and 2 (survival and subsequent cardiac surgeries), predictor variables, and histology of hearts explanted at transplantation. SVD developed in 32 (12%) patients and resolved in 13 (41%); mean age of onset was 3.0 ± 1.63 months; median = 2.79. SVD was not associated with cardiac anatomy, type of stage 1 procedure, weight, coronary abnormality, or atrioventricular valve regurgitation. The mean age of resolution = 12.1 ± 9.6 months; median = 6.3, and resolution may have been more likely with a systemic LV than RV (p = 0.067). Outcomes for the entire SVD group were less favorable than for those without, but patients with resolution of SVD had outcomes at least as good those without SVD. Myocardial histology (n = 4) suggested chronic ischemia. The risk of SVD after stage 1, while low, may be a fundamental feature of this patient population. SVD occurs with either a systemic RV or LV, although patients with a systemic LV may be more likely to have resolution than those with an RV. We identified no predictor variables, but histologic findings suggest chronic ischemia may be involved. Given the low incidence of SVD, multi-center studies will be required to better define predictors of onset and resolution.

Keywords

Hypoplastic left heart syndrome Norwood Sano Cardiomyopathy Ventricular dysfunction 

Notes

Acknowledgements

We acknowledge the expert assistance of Lynne Patkin, MBA, in aiding with preparation of this manuscript.

Compliance with Ethical Standards

Conflict of interest

T. Kulik, L. Sleeper, C. VanderPluym and S. Sanders declares that they have no conflict of interest.

Ethical Approval

All procedures performed in studies involving human participants were is accordance with the ethical standards of the institutional research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. This study was approved by the Institutional Review Board of Boston Children’s Hospital.

References

  1. 1.
    Piran S, Veldtman G, Siu S, Webb GD, Liu PP (2002) Heart failure and ventricular dysfunction in patients with single or systemic right ventricles. Circulation 105(10):1189–1194CrossRefGoogle Scholar
  2. 2.
    Hsu DT, Zak V, Mahony L, 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, Pediatric Heart Network Investigators (2010) Enalapril in infants with single ventricle: results of a multicenter randomized trial. Circulation 122(4):333–340.  https://doi.org/10.1161/CIRCULATIONAHA.109.927988 CrossRefPubMedPubMedCentralGoogle Scholar
  3. 3.
    Khoo NS, Smallhorn JF, Kaneko S, Myers K, Kutty S, Tham EB (2011) Novel insights into RV adaptation and function in hypoplastic left heart syndrome between the first 2 stages of surgical palliation. JACC Cardiovasc Imaging 4(2):128–137.  https://doi.org/10.1016/j.jcmg.2010.09.022 CrossRefPubMedGoogle Scholar
  4. 4.
    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(2):239–247.  https://doi.org/10.1007/s00246-015-1268-4 CrossRefPubMedGoogle Scholar
  5. 5.
    Walsh MA, McCrindle BW, Dipchand A, Manlhiot C, Hickey E, Caldarone CA, Van Arsdell GS, Schwartz SM (2009) Left ventricular morphology influences mortality after the Norwood operation. Heart 95(15):1238–1244.  https://doi.org/10.1136/hrt.2008.156612 CrossRefPubMedGoogle Scholar
  6. 6.
    Sugiyama H, Yutani C, Iida K, Arakaki Y, Yamada O, Kamiya T (1999) The relation between right ventricular function and left ventricular morphology in hypoplastic left heart syndrome: angiographic and pathological studies. Pediatr Cardiol 20(6):422–427.  https://doi.org/10.1007/s002469900504 CrossRefPubMedGoogle Scholar
  7. 7.
    Theis JL, Zimmermann MT, Evans JM, Eckloff BW, Wieben ED, Qureshi MY, O’Leary PW, Olson TM (2015) Recessive MYH6 mutations in hypoplastic left heart with reduced ejection fraction. Circ Cardiovasc Genetics 8(4):564–571.  https://doi.org/10.1161/CIRCGENETICS.115.001070 CrossRefGoogle Scholar
  8. 8.
    Rahimtoola SH (1989) The hibernating myocardium. American Heart J 117(1):211–221CrossRefGoogle Scholar
  9. 9.
    Briceno N, Schuster A, Lumley M, Perera D (2016) Ischaemic cardiomyopathy: pathophysiology, assessment and the role of revascularisation. Heart 102(5):397–406.  https://doi.org/10.1136/heartjnl-2015-308037 CrossRefPubMedGoogle Scholar
  10. 10.
    Aiyagari R, Rhodes JF, Shrader P, Radtke WA, Bandisode VM, Bergersen L, Gillespie MJ, Gray RG, Guey LT, Hill KD, Hirsch R, Kim DW, Lee KJ, Pelech AN, Ringewald J, Takao C, Vincent JA, Ohye RG, Pediatric Heart Network Investigators (2014) Impact of pre-stage II hemodynamics and pulmonary artery anatomy on 12-month outcomes in the Pediatric Heart Network Single Ventricle Reconstruction trial. J Thorac Cardiovasc Surg 148(4):1467–1474.  https://doi.org/10.1016/j.jtcvs.2013.10.057 CrossRefPubMedGoogle Scholar
  11. 11.
    Cole CR, Eghtesady P (2016) The myocardial and coronary histopathology and pathogenesis of hypoplastic left heart syndrome. Cardiol Young 26(1):19–29.  https://doi.org/10.1017/S1047951115001171 CrossRefPubMedGoogle Scholar
  12. 12.
    Salih C, Sheppard MN, Ho SY (2004) Morphometry of coronary capillaries in hypoplastic left heart syndrome. Ann Thorac Surg 77(3):903–907.  https://doi.org/10.1016/j.athoracsur.2003.07.046 (discussion 907)CrossRefPubMedGoogle Scholar
  13. 13.
    Sathanandam S, Cui W, Nguyen NV, Husayni TS, Van Bergen AH, Sajan I, El-Zein C, Polimenakos A, Ilbawi MN, Roberson DA (2010) Ventriculocoronary artery connections with the hypoplastic left heart: a 4-year prospective study: incidence, echocardiographic and clinical features. Pediatr Cardiol 31(8):1176–1185.  https://doi.org/10.1007/s00246-010-9783-9 CrossRefPubMedGoogle Scholar
  14. 14.
    Hummel J, Stiller B, Grohmann J (2013) Coil-occlusion of the left ventricle as emergency treatment in failing stage I palliation for hypoplastic left heart syndrome with sinusoids. Catheter Cardiovasc Interv 81(1):119–124.  https://doi.org/10.1002/ccd.24354 CrossRefPubMedGoogle Scholar
  15. 15.
    Yoshitake S, Miyamoto T, Tanaka Y, Naito Y (2016) Two cases of ventriculocoronary connection from the left ventricle in hypoplastic left heart syndrome. Ann Thorac Surg 101(3):1190–1193.  https://doi.org/10.1016/j.athoracsur.2015.04.048 CrossRefPubMedGoogle Scholar
  16. 16.
    Fogel MA, Rychik J, Vetter J, Donofrio MT, Jacobs M (1997) Effect of volume unloading surgery on coronary flow dynamics in patients with aortic atresia. J Thorac Cardiovasc Surg 113(4):718–726.  https://doi.org/10.1016/S0022-5223(97)70229-0 (discussion 726–717).CrossRefPubMedGoogle Scholar
  17. 17.
    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(1):103–110CrossRefGoogle Scholar
  18. 18.
    Saiki H, Kuwata S, Kurishima C, Masutani S, Senzaki H (2016) Vulnerability of coronary circulation after norwood operation. Ann Thorac Surg 101(4):1544–1551.  https://doi.org/10.1016/j.athoracsur.2015.10.077 CrossRefPubMedGoogle Scholar
  19. 19.
    Topol EJ, Weiss JL, Guzman PA, Dorsey-Lima S, Blanck TJ, Humphrey LS, Baumgartner WA, Flaherty JT, Reitz BA (1984) Immediate improvement of dysfunctional myocardial segments after coronary revascularization: detection by intraoperative transesophageal echocardiography. J Am Coll Cardiol 4(6):1123–1134CrossRefGoogle Scholar
  20. 20.
    Samady H, Elefteriades JA, Abbott BG, Mattera JA, McPherson CA, Wackers FJ (1999) Failure to improve left ventricular function after coronary revascularization for ischemic cardiomyopathy is not associated with worse outcome. Circulation 100(12):1298–1304CrossRefGoogle Scholar
  21. 21.
    Margossian R, Schwartz ML, Prakash A, Wruck L, Colan SD, Atz AM, Bradley TJ, Fogel MA, Hurwitz LM, Marcus E, Powell AJ, Printz BF, Puchalski MD, Rychik J, Shirali G, Williams R, Yoo SJ, Geva T, Pediatric Heart Network Investigators (2009) Comparison of echocardiographic and cardiac magnetic resonance imaging measurements of functional single ventricular volumes, mass, and ejection fraction (from the Pediatric Heart Network Fontan Cross-Sectional Study). Am J Cardiol 104(3):419–428.  https://doi.org/10.1016/j.amjcard.2009.03.058 CrossRefPubMedPubMedCentralGoogle Scholar
  22. 22.
    Bellsham-Revell HR, Simpson JM, Miller OI, Bell AJ (2013) Subjective evaluation of right ventricular systolic function in hypoplastic left heart syndrome: how accurate is it? J Am Soc Echocardiogr 26(1):52–56.  https://doi.org/10.1016/j.echo.2012.09.020 CrossRefPubMedPubMedCentralGoogle Scholar
  23. 23.
    Altmann K, Printz BF, Solowiejczky DE, Gersony WM, Quaegebeur J, Apfel HD (2000) Two-dimensional echocardiographic assessment of right ventricular function as a predictor of outcome in hypoplastic left heart syndrome. Am J Cardiol 86(9):964–968CrossRefGoogle Scholar

Copyright information

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

Authors and Affiliations

  1. 1.Department of CardiologyBoston Children’s HospitalBostonUSA
  2. 2.Division of Cardiac Critical CareBoston Children’s HospitalBostonUSA
  3. 3.The Pulmonary Hypertension ProgramBoston Children’s HospitalBostonUSA
  4. 4.Harvard Medical SchoolBoston Children’s HospitalBostonUSA

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