The Interplay between Myocardial Fibrosis, Strain Imaging and Collagen Biomarkers in Adults with Repaired Tetralogy of Fallot.

Konstantina Karali,Constantinos H Davos,Haralambos Karvounis,Antonios Ziakas,Theodoros D Karamitsos,Alexandros Kallifatidis,George Giannakoulas,Kali Makedou,Matthaios Didagelos,Stavros Hadjimiltiades

doi:10.3390/diagnostics11112101

Abstract

Background: We sought to assess the interplay between right ventricle (RV) fibrosis, biventricular dysfunction based on global longitudinal strain (GLS) analysis, and biomarkers such as Galectin-3 (Gal-3), procollagen type III (PCIII), and NTproBNP. Methods: We studied 35 adult patients with rToF. All patients underwent a cardiac magnetic resonance (CMR) scan including feature tracking for deformation imaging. Blood biomarkers were measured. Results: LGE RV was detected in all patients, mainly at surgical sites. Patients with the highest RV LGE scoring had greater RV dilatation and dysfunction whereas left ventricular (LV) function was preserved. LV GLS correlated with RV total fibrosis score (p = 0.007). A LV GLS value of −15.9% predicted LGE RV score > 8 (AUC 0.754 (p = 0.02)). Neither RV GLS nor biomarker levels were correlated with the extent of RV fibrosis. A cut-off value for NTproBNP of 145.25 pg/mL predicted LGE RV score > 8 points (AUC 0.729, (p = 0.03)). A cut-off value for Gal-3 of 7.42 ng/mL predicted PR Fraction > 20% [AUC 0.704, (p = 0.05)]. Conclusions: A significant extent of RV fibrosis was mainly detected at surgical sites of RV, affecting RV performance. CMR-FT reveals subtle LV dysfunction in rToF patients, due to decreased performance of the fibrotic RV. Impaired LV function and elevated NTproBNP in rToF reflect a dysfunctional fibrotic RV.

Highlights

The improvement in the management of Tetralogy of Fallot (ToF) has led to the extended survival of adult patients with repaired ToF [1,2,3,4,5,6], and to an increased number of patients with heart failure (HF) who may deteriorate and require hospitalization [7,8]
Background: We sought to assess the interplay between right ventricle (RV) fibrosis, biventricular dysfunction based on global longitudinal strain (GLS) analysis, and biomarkers such as Galectin-3 (Gal-3), procollagen type III (PCIII), and NTproBNP
Myocardial damage resulting from the corrective surgery, the postoperative onset of volume overload or pressure conditions, postoperative conduction disturbances, and ventricular interaction are factors that lead to the development of HF in adult patients with repaired ToF (rToF)

Summary

Introduction

The improvement in the management of Tetralogy of Fallot (ToF) has led to the extended survival of adult patients with repaired ToF (rToF) [1,2,3,4,5,6], and to an increased number of patients with heart failure (HF) who may deteriorate and require hospitalization [7,8]. Myocardial damage resulting from the corrective surgery, the postoperative onset of volume overload or pressure conditions, postoperative conduction disturbances, and ventricular interaction are factors that lead to the development of HF in adult patients with rToF. Fibrosis biomarkers that have been found to play an important role in heart failure are increased in adults with rToF [4,5,6]. Analysis of myocardial strain with feature tracking (FT), which is a CMR-based method, is a sensitive measure of regional and global ventricular contractile function, and may contribute to risk stratification of the growing rToF population [12,13,14,15]. Analysis of global CMR-FT strain parameters of RV and left ventricle (LV) and their correlation with extent of RV fibrosis and biomarker levels

Materials and Methods

Patient Population

CMR Imaging Protocol

RV Fibrosis Scoring and Clinical Correlates

CMR-FT Analysis and Biomarkers Levels in Adults with rToF

Limitations