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Fate of the Right Ventricular Outflow Tract Following Valve-Sparing Repair of Tetralogy of Fallot

  • Omar Toubat
    Affiliations
    Division of Cardiac Surgery, Department of Surgery, Keck School of Medicine of USC, University of Southern California, Los Angeles, California

    Heart Institute, Children's Hospital Los Angeles, Los Angeles, California
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  • Winfield J. Wells
    Affiliations
    Division of Cardiac Surgery, Department of Surgery, Keck School of Medicine of USC, University of Southern California, Los Angeles, California

    Heart Institute, Children's Hospital Los Angeles, Los Angeles, California
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  • Vaughn A. Starnes
    Affiliations
    Division of Cardiac Surgery, Department of Surgery, Keck School of Medicine of USC, University of Southern California, Los Angeles, California

    Heart Institute, Children's Hospital Los Angeles, Los Angeles, California
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  • Subramanyan Ram Kumar
    Correspondence
    Address reprint requests to Subramanyan Ram Kumar, MD, PhD, Heart Institute; Children's Hospital Los Angeles; 4650 Sunset Blvd, Mailstop #66; Los Angeles, CA 90027.
    Affiliations
    Division of Cardiac Surgery, Department of Surgery, Keck School of Medicine of USC, University of Southern California, Los Angeles, California

    Heart Institute, Children's Hospital Los Angeles, Los Angeles, California

    Department of Pediatrics, Keck School of Medicine of USC, University of Southern California, Los Angeles, California
    Search for articles by this author
Published:December 22, 2022DOI:https://doi.org/10.1053/j.semtcvs.2022.12.002
      Valve-sparing repair (VSR) of tetralogy of Fallot (TOF) tends to result in higher residual right ventricular outflow tract (RVOT) gradients. We evaluated the progression and clinical implications of RVOT gradients following VSR of TOF. Demographic, clinical, and operative data were retrospectively collected from consecutive TOF patients who underwent VSR at our institution between 01/2010 and 06/2021. RVOT gradient, pulmonary valve annulus (PVA) diameter and Boston Z-scores were recorded from serial echocardiograms. Data are presented as median and interquartile range or number and percentage. A total of 156 children (boys 92, 59%) underwent VSR at 6.5 (4.9-8.4) months of age and 6.6 kg (5.6- 7.7) weight. There was 1 (0.6%) operative mortality. The remaining 155 patients were followed for 69.4 months (4-106.2). RVOT gradient was 2.4m/s (1.7-2.9) at discharge. It transiently increased, then declined and stabilized during follow-up. PVA Z-score was -1.7 (-3.1 to 0.5) at discharge and ‘grew’ to -0.8 (-1.7 to 0.4) at last follow-up. Freedom from RVOT re-intervention was 97%, 94% and 91% at 1, 5 and 10-year follow-up. Among 67 (43%) patients with PVA Z-score < -2, a similar RVOT gradient pattern was observed and freedom from RVOT re-intervention was 97%, 95% and 95% at 1, 5 and 8-year follow-up. Following VSR of TOF, RVOT gradients transiently increase and then fall as PVA growth catches up, resulting in durable intermediate outcomes. Patients with PVA Z-score < -2 demonstrated a similar pattern of hemodynamics in the RVOT and excellent freedom from reintervention.

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      Linked Article

      • Commentary: The Many Shades of Gradient after Repair of Tetralogy of Fallot
        Seminars in Thoracic and Cardiovascular Surgery
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          We continue to refine the management of Tetralogy of Fallot (TOF) decades after initial surgical treatment was first described in the 1950s. The focus has shifted from relief of gradient and cyanosis to long-term health of the right ventricle. Pertinent to right ventricular health is afterload and preload conditions on the right ventricle and the duration of adverse loading condition. A expert consensus document produced by the American Association of thoracic surgery outlined patient selection and follow up after valve sparing repair.
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