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Right Ventricular Remodeling in Hypoplastic Left Heart Syndrome is Minimally Impacted by Cardiopulmonary Bypass: A Comparison of Norwood vs. Hybrid

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Abstract

Right ventricular (RV) remodeling in hypoplastic left heart syndrome (HLHS) begins prenatally and continues through staged palliations. However, it is unclear if the most marked observed remodeling post-Norwood is secondary to cardiopulmonary bypass (CPB) exposure or if it is an adaptation intrinsic to the systemic RV. This study aims to determine the impact of CPB on RV remodeling in HLHS. Echocardiograms of HLHS survivors undergoing stage 1 Norwood (n = 26) or Hybrid (n = 20) were analyzed at pre- and post-stage 1, pre- and post-bidirectional cavo-pulmonary anastomosis (BCPA), and pre-Fontan. RV fractional area change (FAC), vector velocity imaging for longitudinal & derived circumferential deformation (global radial shortening (GRS) = peak radial displacement/end-diastolic diameter), and deformation ratio (longitudinal/ circumferential) were assessed. Both groups had similar age, clinical status and functional parameters pre-stage 1. No difference in RV size and sphericity at any stage between groups. RVFAC was normal (> 35%) throughout for both groups. Both Norwood and Hybrid patients had increased GRS (p = 0.0001) post-stage 1 and corresponding unchanged longitudinal strain, resulting in decreased deformation ratio (greater relative RV circumferential contraction), p = 0.0001. Deformation ratio remained decreased in both groups in subsequent stages. Irrespective of timing of the first CPB exposure, both Norwood and Hybrid patients underwent similar RV remodeling, with relative increase in circumferential to longitudinal contraction soon after stage 1 palliation. The observed RV remodeling in HLHS survivors were minimally impacted by CPB.

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Abbreviations

RV:

Right ventricle

HLHS:

Hypoplastic left heart syndrome

CPB:

Cardiopulmonary bypass

BCPA:

Bidirectional cavo-pulmonary anastomosis

FAC:

Fractional area change

DHCA:

Deep hypothermic circulatory arrest

References

  1. Barber G, Helton G, Aglira BA, Chin AJ, Murphy JD, Pigott JD et al (1988) The significance of tricuspid regurgitation in hypoplastic left-heart syndrome. Am Heart J 116:1563–1567

    Article  CAS  PubMed  Google Scholar 

  2. Lin LQ, Conway J, Alvarez S et al (2018) Reduced right ventricular fractional area change, strain, and strain rate before bidirectional cavopulmonary anastomosis is associated with medium-term mortality for children with hypoplastic left heart syndrome. J Am Soc Echocardiogr 31:831–842

    Article  PubMed  Google Scholar 

  3. Colquitt JL, Loar RQ, Morris SA et al (2019) Serial strain analysis identifies hypoplastic left heart syndrome infants at risk for cardiac morbidity and mortality: a pilot study. J Am Soc Echocardiogr 32:643–650

    Article  PubMed  Google Scholar 

  4. Brooks PB, Khoo NS, Mackie AS et al (2012) Right ventricular function in fetal hypoplastic left heart syndrome. J Am Soc Echocardiogr 10:1068–1074

    Article  Google Scholar 

  5. Tham EB, Smallhorn JF, Kaneko S et al (2014) Insights into the evolution of myocardial dysfunction in the functionally single RV between staged palliations using speckle-tracking echocardiography. J Am Soc Echocardiogr 27:314–322

    Article  PubMed  Google Scholar 

  6. Khoo NS, Smallhorn JF, Kaneko S et al (2011) Novel insights into RV adaptation and function in HLHS between the first 2 stages of surgical palliation. J Am Coll Cardiol 4:128–137

    Article  Google Scholar 

  7. Kaneko S, Khoo NS, Smallhorn JF et al (2012) Single RV have impaired systolic and diastolic function compared to those of left ventricular morphology. J Am Soc Echocardiogr 25:1222–1230

    Article  PubMed  Google Scholar 

  8. Tan JL, Prati D, Gatzoulis MA et al (2007) The RV response to high afterload: comparison between atrial switch procedure, CCTGA, and idiopathic pulmonary arterial hypertension. Am Heart J 153:681–688

    Article  PubMed  Google Scholar 

  9. Hauser M, Bengel FM, Hager A et al (2003) Impaired myocardial blood flow and coronary flow reserve of the anatomical right systemic ventricle in patients with ccTGA. Heart 89:1231–1235

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  10. Li Y, Xie M, Wang X et al (2015) Impaired right and left ventricular function in asymptomatic children with repaired tetralogy of Fallot by two-dimensional speckle tracking echocardiography study. Echocardiography 32:135–143

    Article  PubMed  Google Scholar 

  11. Nawaytou HM, Yubbu P, Montero AE et al (2016) Left ventricular rotational mechanics in children after heart transplantation. Circ Cardiovasc Imaging. https://doi.org/10.1161/CIRCIMAGING.116.004848

    Article  PubMed  PubMed Central  Google Scholar 

  12. Rabe-Hesketh S, Skrondal A (2008) Multilevel and longitudinal modeling using Stata, 2nd edn. Stata Press, USA

    Google Scholar 

  13. Hamdan A, Thouet T, Sebastian K et al (2008) Regional right ventricular function and timing of contraction in healthy volunteers evaluated by strain-encoded MRI. J Magn Reson Imaging 28:1379–1385

    Article  PubMed  Google Scholar 

  14. Pettersen E, Helle-Valle T, Edvardsen T et al (2007) Contraction pattern of the systemic RV shift from longitudinal to circumferential shortening and absent global ventricular torsion. J Am Coll Cardiol 49:2450–2456

    Article  Google Scholar 

  15. Moceri P, Bouvier P, Baudouy D et al (2017) Cardiac remodelling amongst adults with various aetiologies of pulmonary arterial hypertension including Eisenmenger syndrome-implications on survival and the role of right ventricular transverse strain. Eur Heart J Cardiovasc Imaging 18:1262–1270

    Article  PubMed  Google Scholar 

  16. Friedberg MK, Silverman NH, Dubin AM et al (2007) RV mechanical dyssynchrony in children with HLHS. J Am Soc Echocardiogr 20:1073–1079

    Article  PubMed  Google Scholar 

  17. Monivas Palomero V, Mingo Santos S, Goirigolzarri Artaza J et al (2016) Two-dimensional speckle tracking echocardiography in heart transplant patients: two-year follow-up of right and left ventricular function. Echocardiography 33:703–713

    Article  PubMed  Google Scholar 

  18. Lopaschuk GD, Collins-Nakai R, Olley M et al (1994) Plasma fatty acid levels in infants and adults after myocardial ischemia. Am Heart J 128:61–67

    Article  CAS  PubMed  Google Scholar 

  19. Kantor P, Dyck J, Lopaschuk G (1999) Fatty acid oxidation in the reperfused ischemic heart. Am J Med Sci 318:3–27

    Article  CAS  PubMed  Google Scholar 

  20. Karimi M, Wang LX, Hammel JM et al (2004) Neonatal vulnerability to ischemia and reperfusion: cardioplegic arrest causes greater myocardial apoptosis in neonatal lambs than in mature lambs. J Thorac Cardiovasc Surg 127:490–497

    Article  PubMed  Google Scholar 

  21. Van Arsdell G, Maharaj G, Tom J et al (2000) What is the optimal age for repair of Tetralogy of Fallot? Circulation 102:123–129

    Article  Google Scholar 

  22. Grotenhuis HB, Ruijsink B, Chetan D et al (2016) Impact of Norwood versus hybrid palliation on cardiac size and function in HLHS. Heart 102:966–974

    Article  CAS  PubMed  Google Scholar 

  23. Fischbach J, Sinzobahamvya N, Haun C et al (2013) Interventions after Norwood procedure: comparison of Sano and modified BT-shunt. Pediatr Cardiol 34:112–118

    Article  PubMed  Google Scholar 

  24. Photiadis J, Sinzobahamvya N, Haun C et al (2012) Does the shunt type determine mid-term outcome after Norwood operation? Eur J Cardio-Thorac Surg 42:209–216

    Article  Google Scholar 

  25. Padalino MA, Castellani C, Toffoli S et al (2008) Pathological changes and myocardial remodelling related to the mode of shunting following surgical palliation for HLHS. Cardiol Young 18:415–422

    Article  PubMed  Google Scholar 

  26. Kutty S, Graney BA, Khoo NS et al (2012) Serial assessment of right ventricular volume and function in surgically palliated HLHS using real-time transthoracic 3D echocardiography. J Am Soc Echocardiogr 25:682–689

    Article  PubMed  Google Scholar 

  27. Piran S (2002) Heart failure and ventricular dysfunction in patients with single or systemic RV. Circulation 105:1189–1194

    Article  PubMed  Google Scholar 

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Acknowledgements

This study was sponsored by Women and Children’s Health Research Institute.

Funding

This study was generously sponsored by Women and Children’s Health Research Institute.

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Authors and Affiliations

  1. Division of Pediatric Cardiology, Stollery Children’s Hospital, 8440 112 St NW, unit 4C2.36, Edmonton, AB, T6G2B7, Canada

    Kandice Mah, Jesus Serrano Lomelin, Timothy Colen, Edythe B. Tham, Lily Lin, Luke Eckersley, Jeffrey F. Smallhorn & Nee Scze Khoo

  2. Mazankowski Alberta Heart Institute, Alberta Health Services, 11220 83 Ave NW, Edmonton, AB, T6G 2B7, Canada

    Harald Becher

  3. Labatt Family Heart Centre, The Hospital for Sick Children, 555 University Ave, Toronto, ON, M5G1X8, Canada

    Luc Mertens

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  1. Kandice Mah
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  2. Jesus Serrano Lomelin
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  3. Timothy Colen
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  4. Edythe B. Tham
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  5. Lily Lin
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  7. Jeffrey F. Smallhorn
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  8. Harald Becher
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  9. Luc Mertens
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  10. Nee Scze Khoo
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Corresponding author

Correspondence to Nee Scze Khoo.

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The authors declare that they have no competing interest.

Ethical Approval

His retrospective chart review study involving human participants was in accordance with the ethical standards of the institutional and national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. The research ethics boards at the University of Toronto and Alberta approved this study.

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This study is retrospective and was approved by both institutions research ethics boards.

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Mah, K., Serrano Lomelin, J., Colen, T. et al. Right Ventricular Remodeling in Hypoplastic Left Heart Syndrome is Minimally Impacted by Cardiopulmonary Bypass: A Comparison of Norwood vs. Hybrid. Pediatr Cardiol 42, 294–301 (2021). https://doi.org/10.1007/s00246-020-02482-0

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  • DOI: https://doi.org/10.1007/s00246-020-02482-0

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