Abstract
Purpose
Cystic fibrosis transmembrane conductance regulator is expressed in myocardium, but cardiac involvement in cystic fibrosis (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.
Methods
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.
Results
Stable CF was associated with left ventricular dilatation and hypertrophy (LVH; left ventricular mass: CF 59±9g/m2 vs. control 50±8g/m2; p=0.028). LVH was predominantly driven by extracellular myocardial matrix expansion (extracellular matrix mass: CF 27.5±3.4g vs. control 23.6±5.2g; 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).
Conclusion
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.
Cystic fibrosis transmembrane conductance regulator is expressed in myocardium, but cardiac involvement in cystic fibrosis (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.
Methods
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.
Results
Stable CF was associated with left ventricular dilatation and hypertrophy (LVH; left ventricular mass: CF 59±9g/m2 vs. control 50±8g/m2; p=0.028). LVH was predominantly driven by extracellular myocardial matrix expansion (extracellular matrix mass: CF 27.5±3.4g vs. control 23.6±5.2g; 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).
Conclusion
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.
| Original language | English |
|---|---|
| Journal | The International Journal of Cardiovascular Imaging |
| Publication status | Accepted/In press - 11 Dec 2021 |