Cardiac involvement in cystic fibrosis evaluated using cardiopulmonary magnetic resonance

Jakub Lagan,Charlie Palmer,Erik B Schelbert,David Clark,Joshua Bradley,Christopher A Miller,Christien Fortune,Josephine H Naish,Matthias Schmitt,Rowland Bright-Thomas

doi:10.1007/s10554-021-02496-6

Abstract

Cystic fibrosis (CF) transmembrane conductance regulator is expressed in myocardium, but cardiac involvement in CF remains poorly understood. The recent development of a combined cardiopulmonary magnetic resonance imaging technology allows for a simultaneous interrogation of cardiac and pulmonary structure and function. The aim of this study was to investigate myocardial manifestations in adults with CF, both in a stable state and during an acute respiratory exacerbation, and to investigate the relationship between cardiac and pulmonary disease. Healthy adult volunteers (n = 12) and adults with CF (n = 10) were studied using a multiparametric cardiopulmonary magnetic resonance protocol. CF patients were scanned during an acute respiratory exacerbation and re-scanned when stable. Stable CF was associated with left ventricular dilatation and hypertrophy (LVH; left ventricular mass: CF 59 ± 9 g/m2 vs. control 50 ± 8 g/m2; p = 0.028). LVH was predominantly driven by extracellular myocardial matrix expansion (extracellular matrix mass: CF 27.5 ± 3.4 g vs. control 23.6 ± 5.2 g; p = 0.006; extracellular volume [ECV]: CF 27.6 [24.7–29.8]% vs. control 24.8 [22.9–26.0]%; p = 0.030). Acute CF was associated with an acute reduction in left ventricular function (ejection fraction: acute 57 ± 3% vs. stable 61 ± 5%; p = 0.025) and there was a suggestion of myocardial oedema. Myocardial oedema severity was strongly associated with the severity of airflow limitation (r = − 0.726, p = 0.017). Multiparametric cardiopulmonary magnetic resonance technology allows for a simultaneous interrogation of cardiac and pulmonary structure and function. Stable CF is associated with adverse myocardial remodelling, including left ventricular systolic dilatation and hypertrophy, driven by myocardial fibrosis. CF exacerbation is associated with acute myocardial contractile dysfunction. There is also a suggestion of myocardial oedema in the acute period which is related to pulmonary disease severity.

Highlights

Cystic fibrosis (CF), the most common lethal autosomal recessive disorder in Caucasian populations, is a multisystem condition caused by mutations in the CF transmembrane conductance regulator (CFTR) gene [1]
We demonstrate that the identified left ventricular dilatation and hypertrophy (LVH) in CF is predominantly driven by myocardial fibrosis rather than cellular hypertrophy
Cardiopulmonary Magnetic resonance imaging (MRI) technology allows for simultaneous interrogation of cardiac and pulmonary structure and function, and evaluates their relationship

Summary

Introduction

Cystic fibrosis (CF), the most common lethal autosomal recessive disorder in Caucasian populations, is a multisystem condition caused by mutations in the CF transmembrane conductance regulator (CFTR) gene [1]. Pulmonary manifestations remain the main cause of morbidity and mortality and forced expiratory volume in one second (FEV1) is the strongest indicator of prognosis [1]. CFTR is expressed in myocardium [2], but while cor pulmonale is well recognised, the heart is not generally considered part of the primary disease expression. Existing data regarding cardiac manifestations are conflicting and it is unclear whether cardiac abnormalities, if present, are related to disease severity [3,4,5]. With improving life expectancy, understanding cardiac involvement in CF is important in terms of quality of life, prognosis and transplant candidacy [6]

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