High Variability of Right Ventricular Volumes and Function in Adults with Severe Pulmonary Regurgitation Late After Tetralogy of Fallot Repair

Matthias Greutmann,Javier Ruperti,Fabienne Schwitz,Nora Haag,Bruno Santos Lopes,Lukas Meier,Daniela Babic,Emanuela Valsangiacomo Buechel,Christian Kellenberger,Francesca Bonassin,Christine Attenhofer Jost,Markus Schwerzmann,Kerstin Wustmann,Daniel Tobler

doi:10.1016/j.amjcard.2021.11.022

Abstract

Our aim was to assess changes of right ventricular end-diastolic volumes (RVEDVi) and right ventricular ejection fraction (RVEF) in asymptomatic adults with repaired tetralogy of Fallot, with native right ventricular outflow tract and severe pulmonary regurgitation by serial cardiac magnetic resonance imaging (CMR). The study included 23 asymptomatic adults who underwent ≥3 CMR studies (total of 88 CMR studies). We compared changes in RVEDVi and RVEF between first and last study (median follow-up: 8.8years, interquartile range: 6.3 to 13.1 years) and between all study pairs. Variability of measurements between study pairs (65 consecutive and 139 nonconsecutive CMR study pairs) were assessed using Bland-Altman analysis and intraclass correlation coefficients. On average, there were no significant changes of RVEDVi or RVEF over the study period (change in RVEDVi: +0.4 ± 17.8ml/m2, change in RVEF: -1.0 ± 5.5%). Assessment of variability of measurements between study pairs demonstrated no systematic change in RVEDVi and RVEF between study pairs with limits of agreement within the range of previously published studies (RVEDVi -29.1 to +27.2ml/m2; RVEF -11.5% to 10.2%). High intraclass correlation coefficients for RVEDVi (0.943, 95% CI 0.906 to 0.965, p <0.001) and RVEF (0.815, 95% CI 0.697 to 0.887, p <0.0001) indicate high reliability of reported measurements. In conclusion, in asymptomatic adults with repaired tetralogy of Fallot with native right ventricular outflow tracts and severe pulmonary regurgitation, CMR measurements of RV volumes and RVEF remain stable during follow-up with variability between CMR studies in individual patients, as expected for interobserver and interstudy variability. Measurements derived from a single CMR study or changes occurring between 2 CMR studies should be used with caution for clinical decision-making.

Highlights

Imaging (CMR) has emerged as the reference method for measurements of right ventricular volumes and function
From the Swiss SACHER-registry (ClinicalTrials.gov Identifier NCT 2,258,724), we identified asymptomatic adults with repaired tetralogy of Fallot; severe residual pulmonary regurgitation and a native right ventricular outflow tract; followed at the university hospitals of Basel, Berne, and Zurich (CMRs performed at the University Children’s hospital); who had undergone ≥3 cardiac magnetic resonance imaging (CMR) studies during adulthood; without any interventions, endocarditis, or arrhythmias between studies
Of all adults with repaired tetralogy of Fallot followed at the participating centers, 235 had undergone at least 1 CMR study

Summary

Introduction

Imaging (CMR) has emerged as the reference method for measurements of right ventricular volumes and function. Based on CMR studies, European and North American guidelines for the management of adults with congenital heart disease recommend specific thresholds of right ventricular volumes and function (right ventricular end-diastolic volumes [RVEDVi] of ≥160 ml/m2, right ventricular end-systolic volume [RVESVi] ≥80 ml/m2, right ventricular ejection fraction [RVEF]

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