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Finding the Optimal Timing for Repair of Standard Tetralogy of Fallot: Analysis of Cardiac Magnetic Resonance and Echocardiography Parameters Related to Intermediate Term Outcomes in a Pediatric Population

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Abstract

Introduction

Right ventricular (RV) dilatation is the determining prognostic factor in the long-term follow up of patients with repaired Tetralogy of Fallot (TOF). The objective of this study is to analyze whether the results vary depending on the timing of the complete repair and on the surgical technique applied.

Material–Methods

This is a retrospective longitudinal study in which patients with standard TOF were divided into 3 groups depending on their age at surgical repair: group 1 = Early repair (n = 12,1-8 months), group 2 = Late repair (n = 26, > 8 months), and group 3 = Late repair with previous palliative surgery (n = 17, > 8 months). Clinical, echocardiographic and cardiac magnetic resonance (CMR) data from patients that had received complete reparative surgery in our institution from January 2000 to March 2014 were analyzed and compared.

Results

55 patients with echocardiogram and CMR studies (13.39 ± 3.59 years) were reviewed. All patients had at least moderate pulmonary regurgitation (PR). We observed a positive correlation between PR and right ventricular end-diastolic volume (r2 = 0.418; p = 0,004). Group 3 had more severe right ventricular dilatation than patients in groups 1 and 2 (p = 0.001). No differences in right ventricular end-diastolic volume, PR, and pulmonary trunk dimensions were observed between groups 1 and 2. Patients in group 3 had a longer hospital stay.

Conclusions

Although all patients from our cohort had significant PR, age at surgery was not related to RV or pulmonary trunk dilatation. Previous palliative surgery was associated with more severe right ventricular dilatation and longer hospital stays. No differences were observed between early and late repair groups. Our study suggests that postponing TOF repair to a late stage does not improve the degree of PR or long-term morbidity from RV dilatation. Palliative surgery should be avoided if possible.

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Abbreviations

BSA:

Body surface area

CPB:

Cardiopulmonary bypass

CMR:

Cardiac magnetic resonance

ICD:

Implantable cardioverter defibrillator

LV-EF:

Left ventricular ejection fractions

RV-EF:

Right ventricle ejection fraction

PR:

Pulmonary regurgitation

RV:

Right ventricle

RV-COi:

Right ventricular cardiac output index

RV-EDV:

Right ventricle end-diastolic volume

RV-ESV:

Right ventricular end-systolic volume

RV-SVi:

Right ventricular stroke volume index

TOF:

Tetralogy of Fallot

VE:

Ventricular extrasystolia

VT:

Ventricular tachycardia

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Authors and Affiliations

  1. Pediatric Cardiology Department, Sant Joan de Déu Children Hospital, C/Passeig Sant Joan de Deu, s/n, 08950, Esplugues, Barcelona, Spain

    Esther Aurensanz Clemente, Álex Pérez Casares & Joan Sanchez de Toledo

  2. CMR Imaging Department, Sant Joan de Déu Children Hospital, Barcelona, Spain

    Álex Pérez Casares

  3. Intensive Care Unit, Cardiac Institute Quiron Salud Teknon, Barcelona, Spain

    Pablo Ruiz Frontera

  4. Cardiac Surgery Department, Sant Joan de Déu Children Hospital, Barcelona, Spain

    Jose M. Caffarena Calvar

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  1. Esther Aurensanz Clemente
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  2. Álex Pérez Casares
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  3. Pablo Ruiz Frontera
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  4. Jose M. Caffarena Calvar
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Correspondence to Esther Aurensanz Clemente.

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Clemente, E.A., Casares, Á.P., Frontera, P.R. et al. Finding the Optimal Timing for Repair of Standard Tetralogy of Fallot: Analysis of Cardiac Magnetic Resonance and Echocardiography Parameters Related to Intermediate Term Outcomes in a Pediatric Population. Pediatr Cardiol 42, 1324–1333 (2021). https://doi.org/10.1007/s00246-021-02615-z

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  • DOI: https://doi.org/10.1007/s00246-021-02615-z

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