Pediatric Cardiology

, Volume 35, Issue 2, pp 332–343 | Cite as

Fate of Ventricular and Valve Performance Following Early Bidirectional Glenn Procedure After Norwood Operation Controlled for Hypoplastic Left Heart Syndome Anatomic Subtype

  • Anastasios C. PolimenakosEmail author
  • John W. Bokowski
  • Hani S. Ghawi
  • Chawki F. El-Zein
  • Michel N. Ilbawi
Original Article


The Norwood operation (NO) with a right ventricle (RV)-to-pulmonary artery (PA) shunt (NRVPA) is reportedly associated with early hemodynamic advantage. Shunt strategy has been implicated in ventricular function. Outcomes after NRVPA compared with classic procedure as part of a strategy involving early bidirectional Glenn (BDG) procedure were analyzed with reference to RV, tricuspid, and neoaortic valve performance. Between January 2005 and December 2010, 128 neonates with hypoplastic left heart syndrome (HLHS) underwent NO. Controlled for aortic/mitral stenosis (AS–MS) subtype, 28 patients underwent NRVPA (group A), and 26 patients had classic procedure (group B). The patients with a non-HLHS single-ventricle anatomy and those who had undergone a hybrid approach for HLHS were excluded from the study. The mean age at NO was 6.8 ± 3.5 days in group A and 6.9 ± 3.6 days in group B. Transthoracic echocardiographic evaluation (TTE) after NO (TTE-1) at the midinterval between NO and BDG (TTE-2), before BDG (TTE-3), before Fontan (TTE-4), and at the last follow-up evaluation (TTE-5) was undertaken. Cardiac catheterization was used to assess hemodynamic parameters before the Glenn and Fontan procedures. The operative, interstage, and pre-Fontan survival rates for AS–MS after NO were respectively 88.1 % (90.3 % in group A vs. 84.7 % in group B; p = 0.08), 82.5 % (82.7 % in group A vs. 81.8 % in group B; p = 0.9), and 80.7 % (79.5 % in group A vs. 81.8 % in group B; p = 0.9). The median follow-up period was 39.6 months (interquartile range 2.7–4.9 months). The RV global function, mid- and longitudinal indexed dimensions, fractionated area change before BDG (TTE-1, TTE-2, TTE-3) and after BDG (TTE-4, TTE-5), and right ventricular end-diastolic pressure did not differ statistically between the groups (p > 0.05). No statistically significant difference in tricuspid or neoaortic intervention was found between the groups (p > 0.05). Controlled for the AS–MS HLHS subtype, shunt strategy showed no midterm survival or hemodynamic (ventricular or valve) impact. At midterm, the follow-up need for neoaortic or tricuspid valve surgical intervention was not affected by shunt selection. The structural ventricular adaptation after reversal of shunt physiology was irrespective of shunt strategy.


Hypoplastic left heart syndrome Valve Shunt 


  1. 1.
    Anderson JB, Beekman RH, Border WL et al (2009) Lower weight for age z-score adversely affects hospital length of stay after bidirectional Glenn procedure in 100 infants with single ventricle. J Thorac Cardiovasc Surg 138:397–404CrossRefPubMedGoogle Scholar
  2. 2.
    Ashburn DA, McCrindle BW, Tchervenkov CI et al (2003) Outcomes after the Norwood operation in neonates with critical aortic stenosis or aortic valve atresia. J Thorac Cardiovasc Surg 125:1070–1082CrossRefPubMedGoogle Scholar
  3. 3.
    Ballweg JA, Dominguez TE, Ravishankar C et al (2010) Contemporary comparison of the effect of shunt type in hypoplastic left heart syndrome on the hemodynamics and outcome at Fontan completion. J Thorac Cardiovasc Surg 140:537–544CrossRefPubMedGoogle Scholar
  4. 4.
    Barron JD, Brooks A, Stickley J et al (2009) Norwood procedure using a right ventricle-pulmonary artery conduit: comparison of the right-sided versus left-sided conduit position. J Thorac Cardiovasc Surg 138:528–537CrossRefPubMedGoogle Scholar
  5. 5.
    Bradley SM, Simsic JM, Mcquinn TC et al (2004) Hemodynamic status after the Norwood procedure: a comparison of right ventricle to pulmonary artery connection versus modified Blalock Taussig shunt. Ann Thorac Surg 78:933–941CrossRefPubMedGoogle Scholar
  6. 6.
    Elmi M, Hickey EJ, Williams WG et al (2011) Long-term tricuspid valve function after Norwood operation. J Thorac Cardiovasc Surg 142:1341–1347CrossRefPubMedGoogle Scholar
  7. 7.
    Francois K, Bove T, Panzer J et al (2012) Univentricular heart and Fontan staging: analysis of factors impacting on body growth. Eur J Cardiothorac Surg 41:e139–e145CrossRefPubMedGoogle Scholar
  8. 8.
    Friedman KG, Salvin JW, Wypij D et al (2011) Risk factors for failed staged palliation after bidirectional Glenn in infants who have undergone stage one palliation. Eur J Cardiothorac Surg 40:1000–1006PubMedCentralPubMedGoogle Scholar
  9. 9.
    Frommelt PC, Guey LT, Minich LL et al (2012) Does initial shunt type for the Norwood procedure affect echocardiographic measures of cardiac size and function during infancy? The single ventricle trial. Circulation 125:2630–2638PubMedCentralCrossRefPubMedGoogle Scholar
  10. 10.
    Ghanayem NS, Jaquiss RDB, Cava JR et al (2006) Right ventricle-to-pulmonary artery conduit versus Blalock–Taussig shunt: a hemodynamic comparison. Ann Thorac Surg 82:1603–1610CrossRefPubMedGoogle Scholar
  11. 11.
    Graham EM, Atz AM, Bradley SM et al (2007) Does a ventriculotomy have deleterious effects following palliation in the Norwood procedure using a shunt placed from the right ventricle to the pulmonary arteries? Cardiol Young 17:145–150CrossRefPubMedGoogle Scholar
  12. 12.
    Graham EM, Zyblewski SC, Phillips JW et al (2010) Comparison of Norwood shunt types: Do outcomes differ 6 years later? Ann Thorac Surg 90:31–35CrossRefPubMedGoogle Scholar
  13. 13.
    Honjo O, Atlin CR, Mertens L et al (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 142:326–335CrossRefPubMedGoogle Scholar
  14. 14.
    Hughes ML, Shekerdemian LS, Brizard CP et al (2004) Improved early ventricular performance with a right ventricle-to-pulmonary artery conduit in stage 1 palliation for hypoplastic left heart syndrome: evidence of strain Doppler echocardiography. Heart 90:191–194PubMedCentralCrossRefPubMedGoogle Scholar
  15. 15.
    Jaquiss RD, Siehr SL, Ghanayem NS et al (2006) Early cavopulmonary anastomosis after Norwood procedure results in excellent Fontan outcome. Ann Thorac Surg 82:1260–1266CrossRefPubMedGoogle Scholar
  16. 16.
    Lai L, Laussen PC, Cua CL et al (2007) Outcomes after bidirectional Glenn operation: Blalock–Taussig shunt versus right ventricle-to-pulmonary artery conduit. Ann Thorac Surg 83:1768–1773CrossRefPubMedGoogle Scholar
  17. 17.
    Mahle WT, Cohen MS, Spray TL et al (2001) Atrioventricular valve regurgitation in patients with single ventricle: impact of bidirectional cavopulmonary anastomosis. Ann Thorac Surg 72:831–835CrossRefPubMedGoogle Scholar
  18. 18.
    Mair R, Tulzer G, Sames E 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 126:1378–1384CrossRefPubMedGoogle Scholar
  19. 19.
    Ohye RG, Gomez CA, Goldberg CS et al (2004) Tricuspid valve repair in hypoplastic heft heart syndrome. J Thorac Cardiovasc Surg 127:465–472CrossRefPubMedGoogle Scholar
  20. 20.
    Ohye RG, Sleeper LA, Mahony L et al (3010) Comparison of shunt types in the Norwood procedure for single-ventricle lesions. N Engl J Med 362:1980–1992CrossRefGoogle Scholar
  21. 21.
    Polimenakos AC, Sathanandam SK, Husayni TS et al (2011) Hypoplastic left heart syndrome and aortic atresia-mitral stenosis variant: role of myocardial protection strategy and impact of ventriculo-coronary connections after stage I palliation. Pediatr Cardiol 32:929–939CrossRefPubMedGoogle Scholar
  22. 22.
    Pusca SV, Kanter KR, Kirshbom PM et al (2006) Freedom from neoaortic insufficiency: a comparison of classic Norwood and Norwood-Sano procedures. Congenit Heart Dis 1:289–293CrossRefPubMedGoogle Scholar
  23. 23.
    Sano S, Ishino K, Kado H et al (2004) Outcome of right ventricle-to-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome: a multi-institutional study. Ann Thorac Surg 78:1951–1958CrossRefPubMedGoogle Scholar
  24. 24.
    Sathanandam SK, Polimenakos AC, Blair C et al (2010) Hypoplastic left heart syndrome: feasibility study for patients undergoing completion Fontan at or prior to 2 years of age. Ann Thorac Surg 90:821–829CrossRefPubMedGoogle Scholar
  25. 25.
    Stasik CN, Goldberg KS, Bove EL et al (2006) Current outcomes and risk factors for the Norwood procedure. J Thorac Cardiovasc Surg 131:412–417CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  • Anastasios C. Polimenakos
    • 1
    • 3
    Email author
  • John W. Bokowski
    • 2
  • Hani S. Ghawi
    • 2
  • Chawki F. El-Zein
    • 1
    • 2
  • Michel N. Ilbawi
    • 1
    • 2
  1. 1.Division of Pediatric Cardiovascular Surgery, Advocate Hope Children’s HospitalThe Heart Institute for ChildrenOak LawnUSA
  2. 2.Division of Pediatric Cardiovascular Surgery and Thoracic SurgeryRush Center for Congenital and Structural Heart DiseaseChicagoUSA
  3. 3.Division of Pediatric and Congenital Cardiothoracic Surgery, The Heart Center for Children, J. Weis Children’s HospitalGeisinger ClinicDanvilleUSA

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