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Right Ventricular and Outflow Tract Functional Characteristics After Repair of Tetralogy of Fallot with Major Aortopulmonary Collaterals

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Abstract

This study describes right ventricle (RV) characteristics and right ventricle to pulmonary artery (RV-PA) conduit function pre- and post-repair in patients with tetraology of Fallot with major aortopulmonary collaterals (TOF/MAPCAs). We reviewed patients who underwent single-stage, complete unifocalization, and repair of TOF/MAPCAs between 2006 and 2019 with available pre- and early postoperative echocardiograms. For a subset of patients, 6–12 month follow-up echocardiogram was available. RV and left ventricle (LV) characteristics and RV-PA conduit function were reviewed. Wilcoxon signed rank test and McNemar’s test were used. 170 patients were reviewed, 46 had follow-up echocardiograms. Tricuspid valve annular plane systolic excursion (TAPSE) Z-scores were reduced from pre- (Z-score 0.01) to post-repair (Z-score −4.5, p < 0.001), improved but remained abnormal at follow-up (Z-score −4.0, p < 0.001). RV fractional area change (FAC) and LV ejection fraction were not significantly different before and after surgery. Conduit regurgitation was moderate or greater in 11% at discharge, increased to 65% at follow-up. RV-PA conduit failure (severe pulmonary stenosis or severe pulmonary regurgitation) was noted in 61, and 63% had dilated RV (diastolic RV area Z-score > 2) at follow-up. RV dilation correlated with the severe conduit regurgitation (p = 0.018). Longitudinal RV function was reduced after complete repair of TOF/MAPCAs, with decreased TAPSE and preserved FAC and LV ejection fraction. TAPSE improved but did not normalize at follow-up. Severe RV-PA conduit dysfunction was observed prior to discharge in 11% of patients and in 61% at follow-up. RV dilation was common at follow-up, especially in the presence of severe conduit regurgitation.

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Authors and Affiliations

  1. Department of Pediatrics, Lucile Packard Children’s Hospital, Stanford University, Stanford, CA, USA

    Zsofia B. Long, Rajesh Punn, Alisa A. Arunamata, Ritu Asija & Doff B. McElhinney

  2. Clinical and Translational Research Program, Lucile Packard Children’s Hospital, Stanford University, Stanford, CA, USA

    Yulin Zhang & Doff B. McElhinney

  3. Department of Cardiothoracic Surgery, Lucile Packard Children’s Hospital, Stanford University, Stanford, CA, USA

    Michael Ma, Frank L. Hanley & Doff B. McElhinney

  4. Pediatric Cardiology, Stanford University School of Medicine, 750 Welch Road, Suite 325, Palo Alto, CA, 94304-5731, USA

    Zsofia B. Long

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  1. Zsofia B. Long
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  2. Rajesh Punn
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  3. Yulin Zhang
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  4. Alisa A. Arunamata
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  5. Ritu Asija
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  6. Michael Ma
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  7. Frank L. Hanley
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  8. Doff B. McElhinney
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Contributions

ZL and DM wrote the main manuscript. YZ prepared tables I-III and figure 2. RP and AA provided echocardiographic expertise. All authors reviewed the manuscript.

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Correspondence to Zsofia B. Long.

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Long, Z.B., Punn, R., Zhang, Y. et al. Right Ventricular and Outflow Tract Functional Characteristics After Repair of Tetralogy of Fallot with Major Aortopulmonary Collaterals. Pediatr Cardiol 45, 795–803 (2024). https://doi.org/10.1007/s00246-024-03412-0

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