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Surgery Insight: late complications following repair of tetralogy of Fallot and related surgical strategies for management

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From Nature Clinical Practice Cardiovascular Medicine

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Abstract

Biventricular correction of tetralogy of Fallot was devised more than 50 years ago. Current short-term outcomes are excellent. The potential for late complications is, however, an important concern for the growing number of postrepair survivors. Progressive pulmonary valve regurgitation leading to right heart failure and arrhythmia are centrally important problems faced by these patients. New techniques are, however, likely to change the future outcomes for postrepair survivors. These techniques include percutaneous valve replacement, arrhythmia ablation surgery, and strategies that emphasize preservation of the pulmonary valve even at the cost of leaving some residual valvular stenosis. The objectives of this Review are to outline the major complications that arise late after repair of tetralogy of Fallot, to describe the surgical approaches that have been developed to avoid and manage arising complications, and to briefly explore how novel treatment paradigms could change the future long-term outlook for patients following tetralogy repair.

Key Points

  • The outlook for patients with tetralogy of Fallot has dramatically improved in the past 60 years

  • Pulmonary valve regurgitation and arrhythmia are the major problems faced by long-term survivors following repair of tetralogy of Fallot

  • Guidelines for optimum timing of pulmonary valve replacement remain unclear

  • New catheter-based procedures and an emphasis on sparing the pulmonary valve, even at the cost of leaving some residual stenosis, could change postrepair outcomes in the future

  • The interaction between residual hemodynamic lesions and arrhythmia is becoming clearer

  • Long-term studies of survivors following repair of tetralogy of Fallot are required to direct care

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Figure 1: Monology of Fallot.
Figure 2: Kaplan–Meier curves showing improvements in estimated survival for patients with tetralogy of Fallot enrolled in the continuing Global Outcomes Tracking in Congenital Heart Adults study, stratified by decade of birth.
Figure 3: Timing and operative mortality for repair of tetralogy of Fallot at the Hospital for Sick Children, Toronto, ON, Canada.
Figure 4: A risk-adjusted nomogram generated from the multivariable equation for de novo arrhythmia after late reoperation among 174 patients.
Figure 5: Freedom from ventricular and supraventricular tachycardia after late reoperation.

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Authors and Affiliations

  1. current John W Kirklin Fellow of the Division of Cardiovascular Surgery, Congenital Heart Surgeons Society, Hospital for Sick Children and the Toronto Congenital Cardiac Center for Adults, Toronto, ON, Canada

    Tara Karamlou

  2. a professor of surgery in the Division of Cardiovascular Surgery, Hospital for Sick Children and the Toronto Congenital Cardiac Center for Adults, Toronto, ON, Canada

    Brian W McCrindle

  3. a professor of pediatrics in the Division of Cardiology, Hospital for Sick Children, Toronto

    William G Williams

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  1. Tara Karamlou
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  2. Brian W McCrindle
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  3. William G Williams
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Correspondence to William G Williams.

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Karamlou, T., McCrindle, B. & Williams, W. Surgery Insight: late complications following repair of tetralogy of Fallot and related surgical strategies for management. Nat Rev Cardiol 3, 611–622 (2006). https://doi.org/10.1038/ncpcardio0682

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