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Commentary| Volume 161, ISSUE 4, P1435-1436, April 2021

Commentary: How to avoid early Fontan failure?

      Figure thumbnail fx1
      The evaluation of the prospective Fontan candidate requires a multimodality approach.
      Our goal should be to achieve a zero percent rate of early and late Fontan failure.
      See Article page 1426.
      We congratulate Quail and colleagues
      • Quail M.A.
      • Chan I.
      • Sarna S.
      • Hughes M.
      • Muthurangu V.
      A preoperative estimate of central venous pressure is associated with early Fontan failure.
      for their article in this issue of the Journal and their efforts to advance this complex field of single-ventricle palliation. Currently, the Fontan procedure, or total cavopulmonary connection (TCPC), serves as the last step of palliation for several single-ventricle congenital heart conditions.
      • Mery C.M.
      • De León L.E.
      • Trujillo-Diaz D.
      • Ocampo E.C.
      • Dickerson H.A.
      • Zhu H.
      • et al.
      Contemporary outcomes of the Fontan operation: a large single-institution cohort.
      It allows for the entire systemic venous return to passively flow to the pulmonary arterial tree without an interposed pump.
      • Mori M.
      • Aguirre A.J.
      • Elder R.W.
      • Kashkouli A.
      • Farris A.B.
      • Ford R.M.
      • et al.
      Beyond a broken heart: circulatory dysfunction in the failing Fontan.
      Several requirements must be met for TCPC to be successful (some well-known, others still being explored), necessitating extensive and meticulous evaluation of pre-Fontan patients.
      The current common practice of pre-TCPC evaluation is aimed at providing an in-depth physiological understanding of the prospective Fontan candidate, with one of the goals being to avoid, among several other complications, early Fontan failure (EFF). EFF is a devastating scenario following TCPC that presents with low cardiac output and elevated central venous pressure (CVP).
      • Chaudhari M.
      • Sturman J.
      • O'Sullivan J.
      • Smith J.
      • Wrightson N.
      • Parry G.
      • et al.
      Rescue cardiac transplantation for early failure of the Fontan-type circulation in children.
      During this evaluation, patients usually undergo echocardiography, cardiac catheterization, and computed tomography or cardiovascular magnetic resonance (CMR). Although some centers have a wide implementation of preoperative cardiac catheterization,
      • Mery C.M.
      • De León L.E.
      • Trujillo-Diaz D.
      • Ocampo E.C.
      • Dickerson H.A.
      • Zhu H.
      • et al.
      Contemporary outcomes of the Fontan operation: a large single-institution cohort.
      others do not.
      • Rychik J.
      • Atz A.M.
      • Celermajer D.S.
      • Deal B.J.
      • Gatzoulis M.A.
      • Gewillig M.H.
      • et al.
      Evaluation and management of the child and adult with Fontan circulation: a scientific statement from the American Heart Association.
      Cardiac catheterization provides precise hemodynamic and physiological parameters of the patient being evaluated.
      In their study, Quail and colleagues attempted to omit the pre-TCPC catheterization, using a different approach to predict post-TCPC CVP (CVPTCPC). It is generally accepted that elevated CVP after TCPC is associated with Fontan failure.
      • Chaudhari M.
      • Sturman J.
      • O'Sullivan J.
      • Smith J.
      • Wrightson N.
      • Parry G.
      • et al.
      Rescue cardiac transplantation for early failure of the Fontan-type circulation in children.
      The authors used information gathered from a pre-TCPC CMR, along with the CVP measured via jugular venipuncture at the same time, to predict the CVPTCPC.
      Although the authors did not demonstrate a perfect correlation between the predicted and actual post-TCPC CVP, they did find a consistent association between the predicted CVPTCPC and EFF. They concluded that estimated CVPTCPC can help guide important clinical decisions, such as creating a preemptive TCPC fenestration (reserved by some institutions for high-risk patients).
      • Salazar J.D.
      • Zafar F.
      • Siddiqui K.
      • Coleman R.D.
      • Morales D.L.S.
      • Heinle J.S.
      • et al.
      Fenestration during Fontan palliation: now the exception instead of the rule.
      It should be mentioned that the rate of elective TCPC fenestration in this study was rather high (41%).
      The authors should be congratulated for this important contribution to the body of knowledge, and for providing us with additional tools in our everlasting attempt to better define the ideal hemodynamics for Fontan patients. Owing to the critical importance of this palliative step and its potential morbid and/or fatal complications, it is our practice and recommendation to undertake elaborate and comprehensive preoperative evaluation for all patients. We recommend continuing to perform pre-TCPC catheterization, as well as taking a highly targeted approach to TCPC fenestration. The value of CVPTCPC can very well be added to our armamentarium.
      The ultimate goal should be to have an early Fontan failure rate of zero percent.

      References

        • Quail M.A.
        • Chan I.
        • Sarna S.
        • Hughes M.
        • Muthurangu V.
        A preoperative estimate of central venous pressure is associated with early Fontan failure.
        J Thorac Cardiovasc Surg. 2021; 161: 1426-1434
        • Mery C.M.
        • De León L.E.
        • Trujillo-Diaz D.
        • Ocampo E.C.
        • Dickerson H.A.
        • Zhu H.
        • et al.
        Contemporary outcomes of the Fontan operation: a large single-institution cohort.
        Ann Thorac Surg. 2019; 105: 1439-1446
        • Mori M.
        • Aguirre A.J.
        • Elder R.W.
        • Kashkouli A.
        • Farris A.B.
        • Ford R.M.
        • et al.
        Beyond a broken heart: circulatory dysfunction in the failing Fontan.
        Pediatr Cardiol. 2014; 35: 569-579
        • Chaudhari M.
        • Sturman J.
        • O'Sullivan J.
        • Smith J.
        • Wrightson N.
        • Parry G.
        • et al.
        Rescue cardiac transplantation for early failure of the Fontan-type circulation in children.
        J Thorac Cardiovasc Surg. 2005; 129: 416-422
        • Rychik J.
        • Atz A.M.
        • Celermajer D.S.
        • Deal B.J.
        • Gatzoulis M.A.
        • Gewillig M.H.
        • et al.
        Evaluation and management of the child and adult with Fontan circulation: a scientific statement from the American Heart Association.
        Circulation. 2019; 140: e234-e284
        • Salazar J.D.
        • Zafar F.
        • Siddiqui K.
        • Coleman R.D.
        • Morales D.L.S.
        • Heinle J.S.
        • et al.
        Fenestration during Fontan palliation: now the exception instead of the rule.
        J Thorac Cardiovasc Surg. 2010; 140: 129-136

      Linked Article

      • A preoperative estimate of central venous pressure is associated with early Fontan failure
        The Journal of Thoracic and Cardiovascular SurgeryVol. 161Issue 4
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          Early Fontan failure is a serious complication after total cavopulmonary connection, characterized by high central venous pressure, low cardiac output, and resistance to medical therapy. This study aimed to estimate postoperative central venous pressure in patients with total cavopulmonary connection using data routinely collected during preoperative assessment. We sought to determine if this metric correlated with measured postoperative central venous pressure and if it was associated with early Fontan failure.
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