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Influence of Shunt Type on Survival and Right Heart Function after the Norwood Procedure for Aortic Atresia

  • Author Footnotes
    # Both authors contributed equally.
    Nicole Piber
    Footnotes
    # Both authors contributed equally.
    Affiliations
    Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universität München, Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Munich, Germany
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  • Author Footnotes
    # Both authors contributed equally.
    Masamichi Ono
    Correspondence
    Address reprint requests to Masamichi Ono, MD, PhD, Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Lazarettstraße 36, Munich 80636, Germany.
    Footnotes
    # Both authors contributed equally.
    Affiliations
    Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universität München, Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Munich, Germany
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  • Jonas Palm
    Affiliations
    Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Center Munich Technische Universität München, Munich, Germany
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  • Takashi Kido
    Affiliations
    Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universität München, Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Munich, Germany
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  • Melchior Burri
    Affiliations
    Department of Cardiovascular Surgery, German Heart Center Munich, Technische Universität München, Munich, Germany
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  • Christoph Röhlig
    Affiliations
    Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Center Munich Technische Universität München, Munich, Germany
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  • Martina Strbad
    Affiliations
    Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universität München, Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Munich, Germany
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  • Julie Cleuziou
    Affiliations
    Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universität München, Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Munich, Germany
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  • Julia Lemmer
    Affiliations
    Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Center Munich Technische Universität München, Munich, Germany
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  • Daniel Dilber
    Affiliations
    Department of Pediatrics, University Hospital Centre Zagreb, School of medicine Zagreb, Zagreb, Croatia
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  • Frank Klawonn
    Affiliations
    Biostatistics, Helmholtz Center for Infection Research, Braunschweig, Germany

    Department of Computer Science, Ostfalia University, Wolfenbüttel, Germany
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  • Peter Ewert
    Affiliations
    Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Center Munich Technische Universität München, Munich, Germany
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  • Alfred Hager
    Affiliations
    Department of Pediatric Cardiology and Congenital Heart Disease, German Heart Center Munich Technische Universität München, Munich, Germany
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  • Jürgen Hörer
    Affiliations
    Department of Congenital and Pediatric Heart Surgery, German Heart Center Munich, Technische Universität München, Division of Congenital and Pediatric Heart Surgery, University Hospital of Munich, Ludwig-Maximilians-Universität, Munich, Germany
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  • Author Footnotes
    # Both authors contributed equally.
Published:November 25, 2021DOI:https://doi.org/10.1053/j.semtcvs.2021.11.012
      The study objective was to compare the results after Norwood procedure between modified Blalock-Taussig shunt (MBTS) and right ventricle-to-pulmonary artery conduit (RVPAC) according to Sano in patients with hypoplastic left heart syndrome (HLHS) and aortic atresia (AA). A total of 146 neonates with HLHS and AA who underwent the Norwood procedure at our institution between 2001 and 2020 were divided into 2 groups according to shunt type (MBTS or RVPAC). Survival after the Norwood procedure was compared between the groups. Longitudinal right ventricular and tricuspid valve function in each group were evaluated using cubic splines method. RVPAC was performed in 103 patients and MBTS in 43 according to surgeon preference. There were no differences in the 30-day mortality rates (16.5% vs 16.3%, P  = 0.973). Survival at 0.5, 1 and 3 years was 79.6%, 74.6%, and 68.9% in RVPAC and 66.8%, 64.3%, and 58.5% in MBTS (P  =  0.293). Among 23 patients undergoing tricuspid valve procedure, different mechanisms of tricuspid regurgitation were observed between the groups. Longitudinal analysis revealed greater prevalence of late right ventricular dysfunction in RVPAC patients. In 77 patients who completed Fontan procedure, the postoperative N-terminal pro B-type natriuretic peptide value was significantly higher in RVPAC vs MBTS (554 vs 276 ng/L, P  =  0.007). No survival advantage of RVPAC over MBTS was observed in neonates with HLHS and AA undergoing the Norwood procedure. Longitudinal analysis demonstrated a greater prevalence of right ventricular dysfunction and higher N-terminal pro B-type natriuretic peptide values during late follow-up in patients with RVPAC.

      Graphical Abstract

      Keywords

      Abbreviations:

      AA (aortic atresia), AP (antero-posterior), AS (antero-septal), BCPS (bidirectional cavopulmonary shunt), CI (confidence interval), EF (ejection fraction), HLHS (hypoplastic left heart syndrome), IQR (interquartile ranges), MBTS (modified Blalock-Taussig shunt), NT-proBNP (N-terminal pro B-type natriuretic peptide), PA (pulmonary artery), RV (right ventricle), RVPAC (right ventricle to pulmonary artery conduit), S1P (stage I procedure), S2P (stage II procedure), S3P (stage III procedure), SP (septal-posterior), TCPC (total cavopulmonary connection), TR (tricuspid regurgitation), TV (tricuspid valve), zlog-proBNP (age-adjusted zlog value of NT-proBNP)
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      References

        • Newburger J.W.
        • Sleeper L.A.
        • Gaynor J.W.
        • et al.
        Transplant-free survival and interventions at 6 years in the SVR trial.
        Circulation. 2018; 137: 2246-2253
        • Ohye R.G.
        • Schranz D.
        • D'Udekem Y.
        Current therapy for hypoplastic left heart syndrome and related single ventricle lesions.
        Circulation. 2016; 134: 1265-1279
        • Mascio C.E.
        • Irons M.L.
        • Ittenbach R.F.
        • et al.
        Thirty years and 1663 consecutive Norwood procedures: Has survival plateaued?.
        J Thorac Cardiovasc Surg. 2019; 158: 220-229
        • Sano S.
        • Ishino K.
        • Kawada M.
        • et al.
        Right ventricle-pulmonary artery shunt in first-stage palliation of hypoplastic left heart syndrome.
        J Thorac Cardiovasc Surg. 2003; 126: 504-509
        • Pizarro C.
        • Malec E.
        • Maher K.O.
        • et al.
        Right ventricle to pulmonary artery conduit improves outcome after stage I Norwood for hypoplastic left heart syndrome.
        Circulation. 2003; 108: Ii155-Ii160
        • Mair R.
        • Tulzer G.
        • Sames E.
        • et al.
        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. 2003; 126: 1378-1384
        • Ohye R.G.
        • Sleeper L.A.
        • Mahony L.
        • et al.
        Comparison of shunt types in the Norwood procedure for single-ventricle lesions.
        N Engl J Med. 2010; 362: 1980-1992
        • Wilder T.J.
        • McCrindle B.W.
        • Phillips A.B.
        • et al.
        Survival and right ventricular performance for matched children after stage-1 Norwood: Modified Blalock-Taussig shunt versus right-ventricle-to-pulmonary-artery conduit.
        J Thorac Cardiovasc Surg. 2015; 150 (1452.e1441-1448): 1440-1450
        • Ghanayem N.S.
        • Allen K.R.
        • Tabbutt S.
        • et al.
        Interstage mortality after the Norwood procedure: Results of the multicenter single ventricle reconstruction trial.
        J Thorac Cardiovasc Surg. 2012; 144: 896-906
        • Wong J.
        • Lamata P.
        • Rathod R.H.
        • et al.
        Right ventricular morphology and function following stage I palliation with a modified Blalock-Taussig shunt versus a right ventricle-to-pulmonary artery conduit.
        Eur J Cardiothorac Surg. 2017; 51: 50-57
        • 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.
        Pediatric Cardiology. 2013; 34: 354-363
        • Barron D.J.
        • Haq I.U.
        • Crucean A.
        • et al.
        The importance of age and weight on cavopulmonary shunt (stage II) outcomes after the Norwood procedure: Planned versus unplanned surgery.
        J Thorac Cardiovasc Surg. 2017; 154: 228-238
        • Robbers-Visser D.
        • Kapusta L.
        • van Osch-Gevers L.
        • et al.
        Clinical outcome 5 to 18 years after the Fontan operation performed on children younger than 5 years.
        J Thorac Cardiovasc Surg. 2009; 138: 89-95
        • Heck P.B.
        • Müller J.
        • Weber R.
        • et al.
        Value of N-terminal pro brain natriuretic peptide levels in different types of Fontan circulation.
        Eur J Heart Fail. 2013; 15: 644-649
        • Ponikowski P.
        • Voors A.A.
        • Anker S.D.
        • et al.
        2016 ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure.
        Eur J Heart Fail. 2016; 18: 891-975
        • Nir A.
        • Lindinger A.
        • Rauh M.
        • et al.
        NT-Pro-B-type natriuretic peptide in infants and children: Reference values based on combined data from four studies.
        Pediatr Cardiol. 2009; 30: 3-8
        • Palm J.
        • Hoffmann G.
        • Klawonn F.
        • et al.
        Continuous, complete and comparable NT-proBNP reference ranges in healthy children.
        Clin Chem Lab Med. 2020; 58: 1509-1516
        • Ono M.
        • Kido T.
        • Wallner M.
        • et al.
        Comparison of shunt types in the neonatal Norwood procedure for single ventricle.
        Eur J Cardiothorac Surg. 2021; 60 (online ahead of print): 1084-1091
        • Tweddell J.S.
        • Sleeper L.A.
        • Ohye R.G.
        • et al.
        Intermediate-term mortality and cardiac transplantation in infants with single-ventricle lesions: Risk factors and their interaction with shunt type.
        J Thorac Cardiovasc Surg. 2012; 144: 152-159
        • Ono M.
        • Mayr B.
        • Burri M.
        • et al.
        Tricuspid valve repair in children with hypoplastic left heart syndrome: impact of timing and mechanism on outcome.
        Eur J Cardiothorac Surg. 2020; 57: 1083-1090
        • Schreiber C.
        • Kasnar-Samprec J.
        • Hörer J.
        • et al.
        Ring-enforced right ventricle-to-pulmonary artery conduit in Norwood stage I reduces proximal conduit stenosis.
        Ann Thorac Surg. 2009; 88: 1541-1545
        • Schreiber C.
        • Cleuziou J.
        • Cornelsen J.K.
        • et al.
        Bidirectional cavopulmonary connection without additional pulmonary blood flow as an ideal staging for functional univentricular hearts.
        Eur J Cardiothorac Surg. 2008; 34: 550-554
        • Ono M.
        • Kasnar-Samprec J.
        • Hager A.
        • et al.
        Clinical outcome following total cavopulmonary connection: A 20-year single-centre experience.
        Eur J Cardiothorac Surg. 2016; 50: 632-641
        • Norwood W.I.
        • Lang P.
        • Casteneda A.R.
        • et al.
        Experience with operations for hypoplastic left heart syndrome.
        J Thorac Cardiovasc Surg. 1981; 82: 511-519
        • Vida V.L.
        • Bacha E.A.
        • Larrazabal A.
        • et al.
        Surgical outcome for patients with the mitral stenosis–aortic atresia variant of hypoplastic left heart syndrome.
        J Thorac Cardiovasc Surg. 2008; 135: 339-346
        • Glatz J.A.
        • Fedderly R.T.
        • Ghanayem N.S.
        • et al.
        Impact of mitral stenosis and aortic atresia on survival in hypoplastic left heart syndrome.
        Ann Thorac Surg. 2008; 85: 2057-2062
        • Ohye R.G.
        • Gaynor J.W.
        • Ghanayem N.S.
        • et al.
        Design and rationale of a randomized trial comparing the Blalock-Taussig and right ventricle-pulmonary artery shunts in the Norwood procedure.
        J Thorac Cardiovasc Surg. 2008; 136: 968-975
        • Frommelt P.C.
        • Gerstenberger E.
        • Cnota J.F.
        • et al.
        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. 2014; 64: 2026-2035
        • Ruotsalainen H.K.
        • Pihkala J.
        • Salminen J.
        • et al.
        Initial shunt type at the Norwood operation impacts myocardial function in hypoplastic left heart syndrome.
        Eur J Cardiothorac Surg. 2017; 52: 234-240
        • Eindhoven J.A.
        • Van Den Bosch A.E.
        • Jansen P.R.
        • et al.
        The usefulness of brain natriuretic peptide in complex congenital heart disease.
        J Am Coll Cardiol. 2012; 60: 2140-2149
        • Pruetz J.D.
        • Badran S.
        • Dorey F.
        • et al.
        Differential branch pulmonary artery growth after the Norwood procedure with right ventricle-pulmonary artery conduit versus modified Blalock-Taussig shunt in hypoplastic left heart syndrome.
        J Thorac Cardiovasc Surg. 2009; 137: 1342-1348
        • Fiore A.C.
        • Tobin C.
        • Jureidini S.
        • et al.
        A comparison of the modified Blalock-Taussig shunt with the right ventricle-to-pulmonary artery conduit.
        Ann Thorac Surg. 2011; 91: 1479-1484
        • Caspi J.
        • Pettitt T.W.
        • Mulder T.
        • et al.
        Development of the pulmonary arteries after the Norwood procedure: Comparison between Blalock-Taussig shunt and right ventricular-pulmonary artery conduit.
        Ann Thorac Surg. 2008; 86: 1299-1304
        • Garabedian C.P.
        • Joyce J.J.
        • Ross-Ascuitto N.T.
        • et al.
        Innominate artery steal syndrome after stage I palliation for hypoplastic left heart syndrome.
        Pediatr Cardiol. 1998; 19: 458-462
        • Vasko J.S.
        • Tapper R.I.
        • Kilman J.W.
        Cerebral vascular insufficiency after Blalock-Taussig shunts.
        Ann Thorac Surg. 1968; 5: 311-318

      Linked Article

      • Commentary: Are We Any Closer to Understanding the Influence of Shunt Type in The Norwood Procedure?
        Seminars in Thoracic and Cardiovascular SurgeryVol. 34Issue 4
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          Piber and colleagues from Munich present a single-center retrospective review of Sano right ventricle (RV) to pulmonary artery (PA) shunts vs modified Blalock-Taussig (BT) shunts in the Norwood procedure for hypoplastic left heart syndrome (HLHS) and aortic atresia.1 146 neonates with HLHS who underwent the Norwood between 2001 and 2020 were compared according to shunt type. Sano was performed in 103 patients and BT shunt in 43. There were no statistically significant differences in 30 day mortality or after Stage 2.
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