Skip to main content
Log in

Effect of Ventriculotomy on Right-Ventricular Remodeling in Hypoplastic Left Heart Syndrome: A Histopathological and Echocardiography Correlation Study

  • Original Article
  • Published:
Pediatric Cardiology Aims and scope Submit manuscript

Abstract

The objective of this study was to compare histopathological changes in hypoplastic left heart syndrome right ventricles (RV) of patients undergoing Sano and modified Blalock–Taussig (MBT) shunt and correlate them with echocardiographic findings. Myocardial tissue samples were obtained from hearts with Sano or MBT shunts after transplantation or at autopsy. Histologic sections were reviewed manually and by automated digital image analysis. Velocity vector imaging was performed on echocardiogram images obtained before transplant or death. All of these parameters were compared between the Sano and MBT shunt cohorts. A total of 14 specimens (7 Sano and 7 MBT shunt) were studied. Median age at transplant/death of Sano and MBT shunt cohorts was 11 (range 2–41) and 8 months (range 2–200), respectively. All Sano specimens had a scar at ventriculotomy site, and the mean scar area was 6.2 ± 3.3 cm2. Compared with remote RV free wall, myocardium bordering the scar showed increased fibrosis (34 ± 16 % vs. 28 ± 14 %, p = 0.04) and thinning (0.8 ± 0.9 vs. 5.3 ± 0.8 mm; p < 0.001), which did not regress with time. The Sano ventriculotomy site showed significantly decreased velocity, strain, and strain rate compared with the corresponding contralateral segment. No focal scarring or regional hypokinesia was seen in the MBT shunt cohort. This is the first study to demonstrate histopathological features of ventriculotomy-associated RV myocardial scarring and myocardial thinning. The scarred ventriculotomy site showed decreased segmental myocardial deformation after Norwood with Sano shunt.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7

Similar content being viewed by others

References

  1. Bacha EA, Zimmerman FJ, Mor-Avi V, Weinert L, Starr JP, Sugeng L, Lang RM (2004) Ventricular resynchronization by multisite pacing improves myocardial performance in the postoperative single-ventricle patient. Ann Thorac Surg 78:1678–1683

    Article  PubMed  Google Scholar 

  2. Feinstein JA, Benson DW, Dubin AM, Cohen MS, Maxey DM, Mahle WT et al (2012) Hypoplastic left heart syndrome: current considerations and expectations. J Am Coll Cardiol 59(1 Suppl):S1–S42

    Article  PubMed  Google Scholar 

  3. Horer J, Malcic I, Schreiber C, Lange R (2011) False aneurysm origination from the proximal anastomosis of a right ventricular to pulmonary artery shunt following staged repair of hypoplastic left heart syndrome. Interact Cardiovasc Thorac Surg 12(3):487–489

    Article  PubMed  Google Scholar 

  4. Hsu DT, Zak V, Mahony L, Sleeper LA, Atz AM, Levine JC et al (2010) Enalapril in infants with single ventricle: Results of a multicenter randomized trial. Circulation 122(4):333–340

    Article  PubMed  CAS  Google Scholar 

  5. Januszewska K, Kozlik-Feldmann R, Dalla-Pozza R, Greil S, Abicht J, Netz H et al (2011) Right ventricle-to-pulmonary artery shunt related complications after Norwood procedure. Eur J Cardiothorac Surg 40(3):584–590

    PubMed  Google Scholar 

  6. Mair R, Tulzer G, Sames E, Gitter R, Lechner E, Steiner J et al (2003) Right ventricular to pulmonary artery conduit instead of modified blalock-taussig shunt improves postoperative hemodynamics in newborns after the Norwood operation. J Thorac Cardiovasc Surg 126(5):1378–1384

    Article  PubMed  Google Scholar 

  7. Margossian R, Schwartz ML, Prakash A, Wruck L, Colan SD, Atz AM et al (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

    Article  PubMed  Google Scholar 

  8. Menon SC, Minich LL, Casper TC, Puchalski MD, Hawkins JA, Tani LY (2011) Regional myocardial dysfunction following Norwood with right ventricle to pulmonary artery conduit in patients with hypoplastic left heart syndrome. J Am Soc Echocardiogr 24(8):826–833

    Article  PubMed  Google Scholar 

  9. Montgomery J, Henderson M, Ostrowsky J, Karimi M, Hennein H (2010) Repair of ventricular aneurysm after stage I modified Sano-Norwood procedure. Crit Care Nurse 30(2):54–59

    Article  PubMed  Google Scholar 

  10. Ohye RG, Ludomirsky A, Devaney EJ, Bove EL (2004) Comparison of right ventricle to pulmonary artery conduit and modified Blalock-Taussig shunt hemodynamics after the Norwood operation. Ann Thorac Surg 78(3):1090–1093

    Article  PubMed  Google Scholar 

  11. Padalino MA, Castellani C, Toffoli S, Della Barbera M, Milanesi O, Thiene G et al (2008) Pathological changes and myocardial remodelling related to the mode of shunting following surgical palliation for hypoplastic left heart syndrome. Cardiol Young 18(4):415–422

    Article  PubMed  Google Scholar 

  12. Pizarro C, Malec E, Maher KO, Januszewska K, Gidding SS, Murdison KA, et al. (2003) Right ventricle to pulmonary artery conduit improves outcome after stage I Norwood for hypoplastic left heart syndrome. Circulation 108(Suppl 1):II155–II160

    Google Scholar 

  13. Sano S, Ishino K, Kawada M, Arai S, Kasahara S, Asai T et al (2003) Right ventricle-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 126(2):504–549

    Article  PubMed  Google Scholar 

  14. Sano S, Ishino K, Kawada M, Honjo O (2004) Right ventricle-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome. Semin Thorac Cardiovasc Surg Pediatr Cardiol Surg Annu 7:22–31

    Article  Google Scholar 

  15. Shin SH, Hung CL, Uno H, Hassanein AH, Verma A, Bourgoun M et al (2010) Mechanical dyssynchrony after myocardial infarction in patients with left ventricular dysfunction, heart failure, or both. Circulation 121:1096–1103

    Article  PubMed  Google Scholar 

  16. Sluysmans T, Colan SD (2005) Theoretical and empirical derivation of cardiovascular allometric relationships in children. J Appl Physiol 99(2):445–457

    Article  PubMed  Google Scholar 

  17. Takabayashi S, Shimpo H, Yokoyama K, Onoda K, Mitani Y (2007) Reduced regional right ventricular wall motion after transventricular repair of tetralogy of Fallot. J Thorac Cardiovasc Surg 133(6):1656–1658

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Shaji C. Menon.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Menon, S.C., Erickson, L.K., McFadden, M. et al. Effect of Ventriculotomy on Right-Ventricular Remodeling in Hypoplastic Left Heart Syndrome: A Histopathological and Echocardiography Correlation Study. Pediatr Cardiol 34, 354–363 (2013). https://doi.org/10.1007/s00246-012-0462-x

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00246-012-0462-x

Keywords

Navigation