Neo-aortic insufficiency late after staged reconstruction for hypoplastic left heart syndrome: impact of differences in initial palliative procedures

  • Kentaro UmezuEmail author
  • Yorikazu Harada
  • Takahiko Sakamoto
  • Toru Okamura
  • Shizuya Shintomi
  • Kiyohiro Takigiku
  • Satoshi Yasukochi
Original Article


The neo-aortic insufficiency in patients with hypoplastic left heart syndrome is an important sequela. We assessed the risks of the neo-aortic valve deterioration by the difference of initial palliations: Group I underwent primary Norwood (Nw) with systemic-to-pulmonary artery shunt (SPS), Group II underwent bilateral pulmonary artery banding (bPAB) and subsequent Nw with SPS (bPAB-Nw/SPS), Group III underwent bPAB and subsequent Nw with bidirectional Glenn (BDG) procedure (bPAB-Nw/BDG). The neo-aortic valve z score changes over time did not reach statistical significance in all groups (p = 0.43 for Group I, 0.20 for Group II, and 0.30 for Group III). The degree of neo-aortic valve insufficiency did not change significantly over time during this period except for Group III (p = 0.34 for Group I, 0.20 for Group II, and 0.02 for Group III). On the other hand, dimensions of the neo-aortic annulus and degrees of neo-aortic insufficiency did not differ significantly among the 3 groups at any pre-determined time. The presence or absence of incision into the sino-tubular junction at Nw did not affect the late neo-aortic valve z score or insufficiency. These data indicate that the difference of initial palliative procedures does not affect late neo-aortic valve insufficiency in Nw survivors. Because valve failure may develop in longer follow-up, further observation should be conducted.


Hypoplastic left heart syndrome Late neo-aortic insufficiency Norwood operation Bilateral pulmonary artery banding 



None declared.

Compliance with ethical standards

Conflict of interest

None declared.


  1. 1.
    Norwood WI, Lang P, Hansen DD (1983) Physiologic repair of aortic atresia-hypoplastic left heart syndrome. N Engl J Med 308(1):23–26CrossRefGoogle Scholar
  2. 2.
    Sano S, Ishino K, Kawada M, Arai S, Kasahara S, Asai T, Masuda Z-I, Takeuchi M, Ohtsuki S (2003) Right ventricle-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 126(2):504–509 (discussion 509–510) CrossRefGoogle Scholar
  3. 3.
    Gibbs JL, Wren C, 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(6):551–555CrossRefGoogle Scholar
  4. 4.
    Pizarro C, Norwood WI (2003) Pulmonary artery banding before norwood procedure. Ann Thorac Surg 75(3):1008–1010CrossRefGoogle Scholar
  5. 5.
    Galantowicz M, Cheatham JP, Rodeman R (2008) Hybrid approach for hypoplastic left heart syndrome: intermediate results after the learning curve. Ann Thorac Surg 85(6):2063–2070 (discussion 2070–2071) CrossRefGoogle Scholar
  6. 6.
    Cohen MS, Marion BS, McElhinney DB, Robbers-Visser D, Wvd Woerd, Gaynor JW, Spray TL, Wernovsky G (2003) Neo-aortic root dilation and valve regurgitation up to 21 years after staged reconstruction for hypoplastic left heart syndrome. J Am Coll Cardiol 42(3):533–540CrossRefGoogle Scholar
  7. 7.
    Frommelt PC, Guey LT, Minich LL, Bhat M, Bradley TJ, Colan SD, Ensing G, Gorentz J, Heydarian H, John JB, Lai WW, Levine JC, Mahle WT, Miller SG, Ohye RG, Pearson GD, Shirali GS, Wong PC, Cohen MS (2012) Does initial shunt type for the Norwood procedure affect echocardiographic measures of cardiac size and function during infancy? The Single Ventricle Reconstruction Trial. Circulation 125(21):2630–2638CrossRefGoogle Scholar
  8. 8.
    Sakamoto T, Harada Y, Kosaka Y, Umezu K, Yasukochi S, Takigiku K, Matsui H, Inoue N (2011) Second-stage palliation after bilateral pulmonary artery bands for HLHS and its variants-which is better, modified Norwood or Norwood plus bidirectional Glenn? World J Pediatr Congenit Heart Surg 2(4):558–565CrossRefGoogle Scholar
  9. 9.
    Umezu K, Harada Y, Sakamoto T, Maekawa Y, Takigiku K, Yasukochi S (2012) Fontan completion after Norwood with bidirectional Glenn shunt: their haemodynamics and pulmonary artery configuration. Interact CardioVasc Thorac Surg 15(2):204–208CrossRefGoogle Scholar
  10. 10.
    Baba K, Chaturvedi R, Lee KJ, Caldarone CA, Benson LN (2013) Fate of the ductal stent after hybrid palliation for hypoplastic left heart syndrome. Ann Thorac Surg 95(5):1660–1664CrossRefGoogle Scholar
  11. 11.
    Pizarro C, Baffa JM, Derby CD, Krieger PA (2011) Valve-sparing neo-aortic root replacement after Fontan completion for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 141(4):1083–1084CrossRefGoogle Scholar
  12. 12.
    Takabayashi S, Shimpo H, Ozu Y, Yokoyama K, Kajimoto M (2005) A Fontan completion through stage I bilateral pulmonary artery banding for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 130(5):1464–1465CrossRefGoogle Scholar
  13. 13.
    Photiadis J, Sinzobahamvya N, Haun C, Schneider M, Zartner P, Schindler E, Asfour B, Hraska V (2012) Does the shunt type determine mid-term outcome after Norwood operation? Eur J Cardiothorac Surg 42(2):209–2015 (discussion 2015–2016) CrossRefGoogle Scholar
  14. 14.
    Shinkawa T, Chipman C, Holloway J, Tang X, Gossett JM, Imamura M (2017) Single center experience of aortic bypass graft for aortic arch obstruction in children. Heart Vessels 32(1):76–82CrossRefGoogle Scholar
  15. 15.
    Shimizu S, Kawada T, Une D, Shishido T, Kamiya A, Sano S, Sugimachi M (2016) Hybrid stage I palliation for hypoplastic left heart syndrome has no advantage on ventricular energetics: a theoretical analysis. Heart Vessels 31(1):105–113CrossRefGoogle Scholar
  16. 16.
    Schwartz ML, Gauvreau K, del Nido P, Mayer JE, Colan SD (2004) Long-term predictors of aortic root dilation and aortic regurgitation after arterial switch operation. Circulation 110(Suppl II):II-128–II-132Google Scholar
  17. 17.
    Rito ML, Fittipaldi M, Haththotuwa R, Jones TJ, Khan N, Clift P, Brawn WJ, Barron DJ (2015) Long-term fate of the aortic valve after an arterial switch operation. J Thorac Cardiovasc Surg 149(4):1089–1094CrossRefGoogle Scholar
  18. 18.
    Horer J, Hanke T, Stierle U, Takkenberg JJ, Bogers AJ, Hemmer W, Rein JG, Hetzer R, Hubler M, Robinson DR, Sievers HH, Lange R (2009) Neoaortic root diameters and aortic regurgitation in children after the Ross operation. Ann Thorac Surg 88(2):594–600 (discussion 600) CrossRefGoogle Scholar
  19. 19.
    Frigiola A, Varrica A, Satriano A, Giamberti A, Pome G, Abella R, Carminati M, Carlucci C, Ranucci M, Clinical Outcome RG (2010) Neoaortic valve and root complex evolution after Ross operation in infants, children, and adolescents. Ann Thorac Surg 90(4):1278–1285CrossRefGoogle Scholar
  20. 20.
    Fujii Y, Kasahara S, Kotani Y, Takagaki M, Arai S, Otsuki S, Sano S (2011) Double-barrel Damus–Kaye–Stansel operation is better than end-to-side Damus–Kaye–Stansel operation for preserving the pulmonary valve function: the importance of preserving the shape of the pulmonary sinus. J Thorac Cardiovasc Surg 141(1):193–199CrossRefGoogle Scholar
  21. 21.
    Shuhaiber JH, Patel V, Husayni T, El-Zein C, Barth MJ, Ilbawi MN (2006) Repair of symptomatic neoaortic aneurysm after third-stage palliation for hypoplastic left heart syndrome. J Thorac Cardiovasc Surg 131(2):478–479CrossRefGoogle Scholar
  22. 22.
    Frommelt PC, Gerstenberger E, Cnota JF, Cohen MS, Gorentz J, Hill KD, John JB, Levine JC, Lu J, Mahle WT, McCandless RT, Mertens L, Pearson GD, Spencer C, Thacker D, Williams IA, Wong PC, Newburger JW, Investigators Pediatric Heart Network (2014) Impact of initial shunt type on cardiac size and function in children with single right ventricle anomalies before the Fontan procedure: the single ventricle reconstruction extension trial. J Am Coll Cardiol 64(19):2026–2035CrossRefGoogle Scholar
  23. 23.
    Kasnar-Samprec J, Kuhn A, Horer J, Vogt M, Cleuziou J, Lange R, Schreiber C (2012) Unloading of right ventricle by bidirectional superior cavopulmonary anastomosis in hypoplastic left heart syndrome patients promotes remodeling of systemic right ventricle but does not improve tricuspid regurgitation. J Thorac Cardiovasc Surg 144(5):1102–1108 (discussion 1108–1109) CrossRefGoogle Scholar
  24. 24.
    Formigari R, Toscano A, Giardini A, Gargiulo G, Di Donato R, Picchio FM, Pasquini L (2003) Prevalence and predictors of neoaortic regurgitation after arterial switch operation for transposition of the great arteries. J Thorac Cardiovasc Surg 126(6):1753–1759CrossRefGoogle Scholar
  25. 25.
    Jonas RA (1998) The arterial switch operation for transposition of the great arteries. Oper Tech Thorac Cardiovasc Surg 3(4):238–253CrossRefGoogle Scholar
  26. 26.
    Meyer DB, Spray TL (2005) Stage I—the philadelphia approach. Oper Tech Thorac Cardiovasc Surg 10(4):299–312CrossRefGoogle Scholar
  27. 27.
    Tweddell JS (2005) The Norwood procedure with an Innominate artery-to-pulmonary artery shunt. Oper Tech Thorac Cardiovasc Surg 10(2):123–140CrossRefGoogle Scholar
  28. 28.
    Yamauchi S, Kawata H, Iwai S, Kayatani F, Matsuzaka M, Fukuda I, Kishimoto H (2015) Risk factors for semilunar valve insufficiency after the Damus–Kaye–Stansel procedure. Ann Thorac Surg 100(5):1767–1772CrossRefGoogle Scholar
  29. 29.
    Kanzaki T, Yamagishi M, Miyazaki T, Maeda Y, Yaku H (2015) Valve-sparing neoaortic root replacement late after the norwood and fontan procedures. Ann Thorac Surg 99(1):309–312CrossRefGoogle Scholar

Copyright information

© Springer Japan KK, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of Cardiovascular SurgeryNagano Children’s HospitalNaganoJapan
  2. 2.Department of Pediatric CardiologyNagano Children’s HospitalNaganoJapan
  3. 3.Department of Cardiovascular Surgery, The Heart Institute of JapanTokyo Women’s Medical UniversityTokyoJapan

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