World Journal of Pediatrics

, Volume 14, Issue 5, pp 510–519 | Cite as

Surgical outcome after complete repair of tetralogy of Fallot with absent pulmonary valve: comparison between bovine jugular vein-valved conduit and monocusp-valve patch

  • En-Shi Wang
  • Xue-Song Fan
  • Li Xiang
  • Shou-Jun Li
  • Hao ZhangEmail author
Original Article



The prognosis of tetralogy of Fallot with absent pulmonary valve (TOF/APV) without operation is poor. We evaluated the surgical outcome of TOF/APV in a single center.


Twenty-two TOF/APV patients underwent complete surgical correction in our hospital. Right ventricular outflow tract reconstruction was performed using bovine jugular vein (BJV)-valved conduit implantation (n = 10), homograft-valved conduit implantation (n = 2), or monocusp-valve patch (n = 10). Health-related quality of life (QOL) was evaluated during follow-up.


The overall survival at 5 and 10 years was 86.4 ± 7.3% (confidence interval 69.4–97.2%). The survival rates were significantly different between patients with and without bronchial stenosis (40 and 100%, P = 0.0003, log-rank test). The survival of patients aged > 6 months was higher than those ≤ 6 months (100 vs. 40%, P = 0.0003, log-rank test). Patients with BJV-valved conduits had higher systolic gradients from the right ventricle to the pulmonary artery (RV–PA) compared to those with monocusp-valve patches. BJV-valved conduit implantation was a risk factor for post-operative pulmonary-valve stenosis. The QOL score for patients with BJV-valved conduits was lower than those with monocusp-valve patches (P < 0.05). No reoperation was performed during follow-up.


Bronchial stenosis and lower age (≤ 6 months) were the main factors influencing post-operative survival. The use of a BJV-valved conduit was a main reason for RV–PA restenosis; thus, the use of a BJV-valved conduit may increase the need for repeat intervention and decrease the post-operative quality of life.


Bovine jugular vein-valved conduit Bronchial stenosis Pulmonary stenosis Tetralogy of Fallot with absent pulmonary valve 


Author contributions

ESW contributed to the conception and design, data collection, analysis, and interpretation, statistical analysis, writing of the article, and participated in the critical revision of the article and the language correction. XSF contributed to the conception and design, data collection, analysis, and interpretation. ESW and XSF contributed equally to this article. LX participated in data interpretation. SJL contributed to the critical revision of the article and the statistical analysis. HZ contributed to the conception and design, data analysis, critical revision of the article, and language correction. All authors read and approved the final version of the manuscript.


The study was supported by the National Natural Science Foundation of China (81400242 and 81525002) from ESW and HZ, and Program for Distinguished Professor in PUMC from HZ.

Compliance with ethical standards

Ethical approval

The protocol of this study was carried out according to the principles of the Declaration of Helsinki and approved by the Medical Ethics Committee of Cardiovascular Institute and Fuwai Hospital. Written informed consent was obtained from all the participants before enrolment.

Conflict of interest

No financial or nonfinancial benefits have been received or will be received from any party related directly or indirectly to the subject of this article.

Supplementary material

12519_2018_169_MOESM1_ESM.docx (77 kb)
Supplementary material 1 (DOCX 78 kb)


  1. 1.
    Chevers N. A collection of facts illustrative of the morbid conditions of the pulmonary artery, as bearing upon the treatment of cardiac and pulmonary disease. Lond Med Gaz. 1846;38:828–35.Google Scholar
  2. 2.
    Chevers N. The investigation of pulmonary artery diseases. Arch Gen Med. 1847;15:488–508.Google Scholar
  3. 3.
    Rao BN, Anderson RC, Edwards JE. Anatomic variations in the tetralogy of Fallot. Am Heart J. 1971;81:361–71.CrossRefPubMedCentralPubMedGoogle Scholar
  4. 4.
    Lev M, Eckner FA. The pathologic anatomy of tetralogy of Fallot and its variations. Dis Chest. 1964;45:251–61.CrossRefPubMedCentralPubMedGoogle Scholar
  5. 5.
    Yong MS, Yim D, Brizard CP, Robertson T, Bullock A, d’Udekem Y, et al. Long-term outcomes of patients with absent pulmonary valve syndrome: 38 years of experience. Ann Thorac Surg. 2014;97:1671–7.CrossRefPubMedCentralPubMedGoogle Scholar
  6. 6.
    Dunnigan A, Oldham HN, Benson DW Jr. Absent pulmonary valve syndrome in infancy: surgery reconsidered. Am J Cardiol. 1981;48:117–22.CrossRefPubMedCentralPubMedGoogle Scholar
  7. 7.
    Brown JW, Ruzmetov M, Vijay P, Rodefeld MD, Turrentine MW. Surgical treatment of absent pulmonary valve syndrome associated with bronchial obstruction. Ann Thorac Surg. 2006;82:2221–6.CrossRefPubMedCentralPubMedGoogle Scholar
  8. 8.
    Hu R, Zhang H, Xu Z, Liu J, Su Z, Ding W. Late outcomes for the surgical management of absent pulmonary valve syndrome in infants. Interact Cardiovasc Thorac Surg. 2013;16:792–6.CrossRefPubMedCentralPubMedGoogle Scholar
  9. 9.
    Alsoufi B, Williams WG, Hua Z, Cai S, Karamlou T, Chan CC, et al. Surgical outcomes in the treatment of patients with tetralogy of Fallot and absent pulmonary valve. Eur J Cardiothorac Surg. 2007;31:354–9 (discussion 359).CrossRefPubMedCentralPubMedGoogle Scholar
  10. 10.
    Bové T, Demanet H, Wauthy P, Goldstein JP, Dessy H, Viart P, et al. Early results of valved bovine jugular vein conduit versus bicuspid homograft for right ventricular outflow tract reconstruction. Ann Thorac Surg. 2002;74:536–41 (discussion 541).CrossRefPubMedCentralPubMedGoogle Scholar
  11. 11.
    Prior N, Alphonso N, Arnold P, Peart I, Thorburn K, Venugopal P, et al. Bovine jugular vein valved conduit: up to 10 years follow-up. J Thorac Cardiovasc Surg. 2011;141:983–7.CrossRefPubMedCentralPubMedGoogle Scholar
  12. 12.
    Palma G, Mannacio VA, Mastrogiovanni G, Russolillo V, Cioffi S, Mucerino M, et al. Bovine valved venous xenograft in pulmonary position: medium term evaluation of risk factors for dysfunction and failure after 156 implants. J Cardiovasc Surg (Torino). 2011;52:285–91.Google Scholar
  13. 13.
    Corno AF, Qanadli SD, Sekarski N, Artemisia S, Hurni M, Tozzi P, et al. Bovine valved xenograft in pulmonary position: medium-term follow-up with excellent hemodynamics and freedom from calcification. Ann Thorac Surg. 2004;78:1382–8 (discussion 1382-8).CrossRefPubMedCentralPubMedGoogle Scholar
  14. 14.
    Kumar M, Turrentine MW, Rodefeld MD, Bell T, Brown JW. Right ventricular outflow tract reconstruction with a polytetrafluoroethylene monocusp valve: a 20-year experience. Semin Thorac Cardiovasc Surg. 2016;28:463–70.CrossRefPubMedCentralPubMedGoogle Scholar
  15. 15.
    Gundry SR, Razzouk AJ, Boskind JF, Bansal R, Bailey LL. Fate of the pericardial monocusp pulmonary valve for right ventricular outflow tract reconstruction. Early function, late failure without obstruction. J Thorac Cardiovasc Surg. 1994;107:908–12 (discussion 912-3).PubMedPubMedCentralGoogle Scholar
  16. 16.
    Promphan W, Attanawanit S, Wanitkun S, Khowsathit P. The right and left ventricular function after surgical correction with pericardial monocusp in tetralogy of fallot: mid-term result. J Med Assoc Thai. 2002;85(Suppl 4):S1266–74.PubMedPubMedCentralGoogle Scholar
  17. 17.
    Nath DS, Nussbaum DP, Yurko C, Ragab OM, Shin AJ, Kumar SR, et al. Pulmonary homograft monocusp reconstruction of the right ventricular outflow tract: outcomes to the intermediate term. Ann Thorac Surg. 2010;90:42–9.CrossRefPubMedCentralPubMedGoogle Scholar
  18. 18.
    Hu S, Xie Y, Li S, Wang X, Yan F, Li Y, et al. Double-root translocation for double-outlet right ventricle with noncommitted ventricular septal defect or double-outlet right ventricle with subpulmonary ventricular septal defect associated with pulmonary stenosis: an optimized solution. Ann Thorac Surg. 2010;89:1360–5.CrossRefPubMedCentralPubMedGoogle Scholar
  19. 19.
    Williams RV, Minich LL, Shaddy RE, Pagotto LT, Tani LY. Comparison of Doppler echocardiography with angiography for determining the severity of pulmonary regurgitation. Am J Cardiol. 2002;89:1438–41.CrossRefPubMedCentralPubMedGoogle Scholar
  20. 20.
    Varni JW, Seid M, Rode CA. The PedsQL: measurement model for the pediatric quality of life inventory. Med Care. 1999;37:126–39.CrossRefPubMedCentralPubMedGoogle Scholar
  21. 21.
    Kirshbom PM, Kogon BE. Tetralogy of Fallot with absent pulmonary valve syndrome. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu. 2004;7:65–71.CrossRefPubMedCentralPubMedGoogle Scholar
  22. 22.
    Hew CC, Daebritz SH, Zurakowski D, del Nido PI, Mayer JE Jr, Jonas RA. Valved homograft replacement of aneurysmal pulmonary arteries for severely symptomatic absent pulmonary valve syndrome. Ann Thorac Surg. 2002;73:1778–85.CrossRefPubMedCentralPubMedGoogle Scholar
  23. 23.
    Fiore AC, Ruzmetov M, Huynh D, Hanley S, Rodefeld MD, Turrentine MW, et al. Comparison of bovine jugular vein with pulmonary homograft conduits in children less than 2 years of age. Eur J Cardiothorac Surg. 2010;38:318–25.CrossRefPubMedCentralPubMedGoogle Scholar
  24. 24.
    McDonnell BE, Raff GW, Gaynor JW, Rychik J, Godinez RI, DeCampli WM, et al. Outcome after repair of tetralogy of Fallot with absent pulmonary valve. Ann Thorac Surg. 1999;67:1391–5 (discussion 1395-6).CrossRefPubMedCentralPubMedGoogle Scholar
  25. 25.
    Chen JM, Glickstein JS, Margossian R, Mercando ML, Hellenbrand WE, Mosca RS, et al. Superior outcomes for repair in infants and neonates with tetralogy of Fallot with absent pulmonary valve syndrome. J Thorac Cardiovasc Surg. 2006;132:1099–104.CrossRefPubMedCentralPubMedGoogle Scholar
  26. 26.
    Nørgaard MA, Alphonso N, Newcomb AE, Brizard CP, Cochrane AD. Absent pulmonary valve syndrome. Surgical and clinical outcome with long-term follow-up. Eur J Cardiothorac Surg. 2006;29:682–7.CrossRefPubMedCentralPubMedGoogle Scholar
  27. 27.
    Lee C, Lee CH, Kwak JG, Kim SH, Shim WS, Lee SY, et al. Factors associated with right ventricular dilatation and dysfunction in patients with chronic pulmonary regurgitation after repair of tetralogy of Fallot: analysis of magnetic resonance imaging data from 218 patients. J Thorac Cardiovasc Surg. 2014;148:2589–95.CrossRefPubMedCentralPubMedGoogle Scholar
  28. 28.
    Breymann T, Blanz U, Wojtalik MA, Daenen W, Hetzer R, Sarris G, et al. European Contegra multicentre study: 7-year results after 165 valved bovine jugular vein graft implantations. Thorac Cardiovasc Surg. 2009;57:257–69.CrossRefPubMedCentralPubMedGoogle Scholar
  29. 29.
    Uzark K, Griffin L, Rodriguez R, Zamberlan M, Murphy P, Nasman C, et al. Quality of life in pediatric heart transplant recipients: a comparison with children with and without heart disease. J Heart Lung Transplant. 2012;31:571–8.CrossRefPubMedCentralPubMedGoogle Scholar

Copyright information

© Children's Hospital, Zhejiang University School of Medicine 2018

Authors and Affiliations

  • En-Shi Wang
    • 1
  • Xue-Song Fan
    • 2
  • Li Xiang
    • 1
  • Shou-Jun Li
    • 1
  • Hao Zhang
    • 1
    Email author
  1. 1.Center for Pediatric Cardiac Surgery, National Center for Cardiovascular Diseases and Fuwai HospitalChinese Academy of Medical Sciences and Peking Union Medical CollegeBeijingChina
  2. 2.Department of Clinical Laboratory Center, Beijing An Zhen HospitalCapital Medical University, Beijing Institute of Heart, Lung and Blood Vessel DiseasesBeijingChina

Personalised recommendations