Skip to main content
SpringerLink
Log in

Obstructive Left Heart Disease in Neonates With a “Borderline” Left Ventricle: Diagnostic Challenges to Choosing the Best Outcome

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

Abstract

In most newborns with left heart obstruction, the choice between a single-ventricle or biventricular management pathway is clear. However, in some neonates with a “borderline” left ventricle, this decision is difficult. Existing criteria do not reliably identify neonates who will have a good long-term outlook after biventricular repair (BVR). The objective of this study was prospective assessment of the outcome after BVR for newborns in whom the left ventricle (LV) was considered “borderline” by an expert group. This study was a prospective follow-up evaluation of neonates with obstructive left heart disease related to a “borderline” LV who underwent biventricular management between January 2005 and April 2011. Of 154 neonates who required intervention for left heart obstruction, 13 (7.8 %) met the echocardiographic (echo) inclusion criteria. At the first and last echo, the z-scores were respectively −1.76 ± 1.37 and −0.66 ± 1.47 (p = 0.013) for the mitral valve, −1.02 ± 1.57 and −0.23 ± 1.78 (p = 0.056) for the aortic valve, and 13.77 ± 5.8 and 20.85 ± 8.9 ml/m2 (p = 0.006) for the LV end-diastolic volume. At this writing, all 12 survivors are clinically well. However, LV diastolic dysfunction and pulmonary artery hypertension was present in 5 (36 %) of 12 patients. Endocardial fibroelastosis (EFE) was detected in five patients at the last follow-up echo, but only in two patients preoperatively. Cardiac magnetic resonance imaging did not confirm EFE in any of assessed patients. The study authors could not reliably predict the outcome after BVR for neonates with left heart obstruction and a “borderline” LV. The presence of EFE with consequent diastolic dysfunction is more important than LV volume in determining the outcome. Prospective identification of EFE remains challenging.

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

Access this article

Log in via an institution

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

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5

Similar content being viewed by others

References

  1. Bacha EA, Daves S, Hardin J, Abdulla R, Anderson J, Kahana M et al (2006) Single-ventricle palliation for high-risk neonates: the emergence of an alternative hybrid stage I strategy. J Thorac Cardiovasc Surg 131:163–171

    Article  PubMed  Google Scholar 

  2. Ballard G, Tibby S, Miller O, Krasemann T, Rosenthal E, Anderson D et al (2010) Growth of left heart structures following the hybrid procedure for borderline hypoplastic left heart. Eur J Echocardiogr 11:870–874

    Article  PubMed  Google Scholar 

  3. Berger M, Haimowitz A, Van Tosh A, Berdoff RL, Goldberg E (1985) Quantitative assessment of pulmonary hypertension in patients with tricuspid regurgitation using continuous wave Doppler ultrasound. J Am Coll Cardiol 6:359–365

    Article  CAS  PubMed  Google Scholar 

  4. Blaufox AD, Lai WW, Lopez L, Nguyen K, Griepp RB, Parness IA (1998) Survival in neonatal biventricular repair of left-sided cardiac obstructive lesions associated with hypoplastic left ventricle. Am J Cardiol 82:1138–1140

    Article  CAS  PubMed  Google Scholar 

  5. Burch M, Kaufman L, Archer N, Sullivan I (2004) Persistent pulmonary hypertension late after neonatal aortic valvotomy: a consequence of an expanded surgicsl cohort. Heart 90:918–920

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  6. Colan SD, McElhinney DB, Crawford EC, Keane JF, Lock JE (2006) Validation and reevaluation of a discriminant model predicting anatomic suitability for biventricular repair in neonates with aortic stenosis. J Am Coll Cardiol 47:1858–1865

    Article  PubMed  Google Scholar 

  7. Corno AF (2005) Borderline left ventricle. Eur J Cardiothorac Surg 27:67–73

    Article  PubMed  Google Scholar 

  8. Cury RC, Abbara S, Sandoval LJ-D, Houser S, Brady TJ, Palacios IF (2005) Visualization of endomyocardial fibrosis by delayed-enhancement magnetic resonance imaging. Circulation 111:e115–e117

    Article  PubMed  Google Scholar 

  9. Eghtesady P, Michelfelder E, Altaye M, Ballard E, Hirsh R, Beekman RH III (2007) Revisiting animal models of aortic stenosis in the early gestation fetus. Ann Thorac Surg 83:631–639

    Article  PubMed  Google Scholar 

  10. Egito ES, Moore P, O’Sullivan J, Colan S, Perry SB, Lock JE et al (1997) Transvascular balloon dilation for neonatal critical aortic stenosis: early and midterm result. J Am Coll Cardiol 29:442–447

    Article  CAS  PubMed  Google Scholar 

  11. Emani SM, Bacha EA, McElhinney DB, Marx GR, Tworetzky W, Pigula FA et al (2009) Primary left ventricular rehabilitation is effective in maintaining two-ventricle physiology in the borderline left heart. J Thorac Cardiovasc Surg 138:1276–1282

    Article  PubMed Central  PubMed  Google Scholar 

  12. Georgiev SG, Eicken A, Balling G, Schreiber C, Hess J (2010) Neonatal balloon aortic valvuloplasty: predictive value ofcurrent risk score algorithms for treatment strategies. Catheter Cardiovasc Interv 76:404–416

    Article  PubMed  Google Scholar 

  13. Gibbs JL, Wren C, Waterson KG, Hunter S, Hamilton JRL (1993) Stenting of the arterial duct combined with banding of the pulmonary arteries and atrial septectomy or septostomy: a new approach to palliation for the hypoplastic left heart syndrome. Br Heart J 69:551–555

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  14. Gutgesell HP, Rembold CM (1990) Growth of the human heart relative to body surface area. Am J Cardiol 65:662–668

    Article  CAS  PubMed  Google Scholar 

  15. Hickey EJ, Caldarone CA, Blackstone EH, Lofland GK, Yeh T, Pizarro C et al (2007) Critical left ventricular outflow tract obstruction: the disproportionate impact of biventricular repair in borderline cases. J Thorac Cardiovasc Surg 134:1429–1437

    Article  PubMed  Google Scholar 

  16. Hraska V, Lilje C, Kantorova A, Photiadis J, Fink C, Asfour B et al (2010) Ross-Konno procedure in children: midterm results. World J Pediatr Congenit Heart Surg 1:28–33

    Article  PubMed  Google Scholar 

  17. Kirklin J, Barratt-Boyes BG (1993) Cardiac Surgery, 2nd edn. Churchill Livingstone, New York, p 1269

    Google Scholar 

  18. Kovalchin JP, Brook MM, Rosenthal GL, Suda K, Hoffman JIE, Silverman NH (1998) Echocardiographic, hemodynamic and morphometric predictors of survival after two-ventricle repair in infants with critical aortic stenosis. J Am Coll Cardiol 32:237–244

    Article  CAS  PubMed  Google Scholar 

  19. Leung MP, McKay R, Smith A, Anderson RH, Arnold R (1991) Critical aortic stenosis in early infancy: anatomic and echocardiographic substrates of successful open valvotomy. J Thorac Cardiovasc Surg 101:526–535

    CAS  PubMed  Google Scholar 

  20. Lofland GK, McCrindle BW, Williams WG, Blackstone EH, Tchervenkov CI, Jonas RA, The Congenital Heart Surgeons’ Society (2001) Critical aortic stenosis in the neonate: a multi-insitutional study of management, outcomes, and risk factors. J Thorac Cardiovasc Surg 121:10–27

    Article  CAS  PubMed  Google Scholar 

  21. Mahle WT, Weinberg PM, Rychik J (1998) Can echocardiography predict the presence or absence of endocardial fibroelastosis in infants <1 year of age with left ventricular outflow obstruction? Am J Cardiol 82:122–124

    Article  CAS  PubMed  Google Scholar 

  22. McElhinney DB, Lock JE, Keane JF, Moran AM, Colan SD (2005) Left heart growth, function, and reintervention after ballooon aortic valvuloplasty for neonatal aortic stenosis. Circulation 111:451–458

    Article  PubMed  Google Scholar 

  23. McElhinney DB, Vogel M, Benson CB, Marshall AC, Wilkins-Haug LE, Silva V et al (2010) Assessment of left ventricular endocardial fibroelastosis in fetuses with aortic stenosis and evolving hypoplastic left heart syndrome. Am J Cardiol 106:1792–1797

    Article  PubMed  Google Scholar 

  24. Mocellin R, Sauer U, Simon B, Comazzi M, Sebening F, Buhlmeyer K (1983) Reduced left ventricular size and endocardial fibroelastosis as correlates of mortality in newborns and young infants with severe aortic valve stenosis. Pediatric Cardiol 4:265–272

    Article  CAS  Google Scholar 

  25. Norwood WI, Kirklin J, Sanders SP (1980) Hypoplastic left heart syndrome: experience with palliative surgery. Am J Cardiol 45:87–91

    Article  CAS  PubMed  Google Scholar 

  26. O’Leary PW, Durongpisitkul K, Cordes TM, Bailey KR, Hagler DJ, Tajik J, Seward JB (1998) Diastolic ventricular function in children: a Doppler echocardiographic study establishing normal values and predictors of increased ventricular end-diastolic pressure. Mayo Clin Proc 73:616–628

    Article  PubMed  Google Scholar 

  27. Oudiz RJ (2005) Cardiac catheterization in pulmonary arterial hypertension: an updated guide to proper use. Adv Pulm Hypertens Autumn 4:15–25

    Google Scholar 

  28. Pettersen MD, Skeens ME, Humes RA (2008) Regression equations for calculation of z-scores of cardiac structures in a large cohort of healthy infants, children, and adolescents: an echocardiographic study. J Am Soc Echocardiogr 21:922–934

    Article  PubMed  Google Scholar 

  29. Rhodes L, Colan S, Perry SB, Jonas RA, Sanders S (1991) Predictors of survival in neonates with critical aortic stenosis. Circulation 84:2325–2335

    Article  CAS  PubMed  Google Scholar 

  30. Salemi VM, Rochitte CE, Shiozaki AA, Andrade JM, Parga JR, de Avila LF et al (2011) Late gadolinium enhancement magnetic resonance imaging in the diagnosis and prognosis of endomyocardial fibrosis patients. Circ Cardiovasc Imaging 4:304–311

    Article  PubMed  Google Scholar 

  31. Schwartz ML, Gauvreau K, Geva T (2001) Predictors of outcome of biventricular repair in infants with multiple left heart obstructive lesions. Circulation 104:682–687

    Article  CAS  PubMed  Google Scholar 

  32. Serraf A, Piot JD, Bonnet N, Lacour-Gayet F, Touchot A, Bruniaux J et al (1999) Biventricular repair approach in duct-dependent neonates with hypoplastic but morphologically normal left ventricle. J Am Coll Cardiol 33:827–834

    Article  CAS  PubMed  Google Scholar 

  33. Shone JD, Sellers RD, Anderson RC, Adams P Jr, Lillehei CW, Edwards JE (1963) The developmental complex of “parachute mitral valve”, supravalvular ring of left atrium, subaortic stenosis, and coarctation of aorta. Am J Cardiol 11:714–725

    Article  CAS  PubMed  Google Scholar 

  34. Stranzinger E, Ensing GJ, Hernandez RJ (2008) MR findings of endocardial fibroelastosis in children. Pediatr Radiol 38:292–296

    Article  PubMed  Google Scholar 

  35. Tani LY, Minich LL, Pagotto LT, Shaddy RE, McGough EC, Hawkins JA (1999) Left heart hypoplasia and neonatal aortic arch obstruction: is the Rhodes left ventricular adequacy score applicable? J Thorac Cardiovasc Surg 118:81–86

    Article  CAS  PubMed  Google Scholar 

  36. Tchervenkov CI, Tahta SA, Jutras LC, Bèland MJ (1998) Biventricular repair in neonates with hypoplastic left heart complex. Ann Thorac Surg 66:1350–1357

    Article  CAS  PubMed  Google Scholar 

  37. Tworetzky W, del Nido PJ, Powell AJ, Marshall AC, Lock JE, Geva T (2005) Usefulness of magnetic resonance imaging of left ventricular endocardial fibroelastosis in infants after fetal intervention for aortic valve steonosis. Am J Cardiol 96:1568–1570

    Article  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Cardiothoracic Unit, Great Ormond Street Hospital for Children, WC1N 3JH, London, UK

    Giulia Tuo, Sachin Khambadkone, Oliver Tann, Martin Kostolny, Graham Derrick, Victor Tsang, Ian Sullivan & Jan Marek

  2. Viale Pio VII 18/4, 16148, Genova, Italy

    Giulia Tuo

  3. Institute of Cardiovascular Science, University College, W1T 7HA, London, UK

    Jan Marek

Authors
  1. Giulia Tuo
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Sachin Khambadkone
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Oliver Tann
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Martin Kostolny
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Graham Derrick
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Victor Tsang
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Ian Sullivan
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Jan Marek
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Giulia Tuo.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Tuo, G., Khambadkone, S., Tann, O. et al. Obstructive Left Heart Disease in Neonates With a “Borderline” Left Ventricle: Diagnostic Challenges to Choosing the Best Outcome. Pediatr Cardiol 34, 1567–1576 (2013). https://doi.org/10.1007/s00246-013-0685-5

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00246-013-0685-5

Keywords

Search

Navigation