title: Heart involvement in cystic fibrosis: A specific cystic fibrosis-related myocardial changes?
abstract: Cystic fibrosis is a complex multi-systemic chronic disease characterized by progressive organ dysfunction with development of fibrosis, possibly affecting the heart. Over the last four decades pathological, experimental, and clinical evidence points towards the existence of a specific myocardial involvement in cystic fibrosis. Multi-modality cardiac imaging, especially recent echocardiographic techniques, evidenced diastolic and/or systolic ventricular dysfunction in cystic fibrosis leading to the concept of a cystic fibrosis-related cardiomyopathy. Hypoxemia and inflammation are among the most important factors for heart involvement in cystic fibrosis. Cystic Fibrosis Transmembrane Regulator was found to be involved in the regulation of cardiomyocyte contraction and may also account for cystic fibrosis-related myocardial dysfunction. This review, mainly focused on echocardiographic studies, seeks to synthesize the existing literature for and against the existence of heart involvement in cystic fibrosis, its mechanisms and prognostic implications. Careful investigation of the heart function may be helpful for risk stratification and therapeutic decisions in patients with cystic fibrosis.
authors: Caen CHU, Department of Cardiology, Caen, F-14000, France. Electronic address: fabien.labombarda@gmail.com.
authors: Fabien
authors: F
authors: Labombarda
authors: Caen CHU, Department of Cardiology, Caen, F-14000, France.
authors: Eric
authors: E
authors: Saloux
authors: Caen CHU, Cystic Fibrosis Center, Department of Pediatrics, Caen, F-14000, France.
authors: Jacques
authors: J
authors: Brouard
authors: Caen CHU, Cystic Fibrosis Center, Department of Pneumology, Caen, F-14000, France.
authors: Emmanuel
authors: E
authors: Bergot
authors: Caen CHU, Department of Cardiology, Caen, F-14000, France.
authors: Paul
authors: P
authors: Milliez
mesh_terms: D009202:Cardiomyopathies / Q000150:complications / Q000503:physiopathology; D003550:Cystic Fibrosis / Q000150:complications* / Q000000981:diagnostic imaging* / Q000473:pathology / Q000503:physiopathology; D004452:Echocardiography / Q000379:methods; D006352:Heart Ventricles / Q000000981:diagnostic imaging* / Q000503:physiopathology; D006801:Humans; D000860:Hypoxia / Q000150:complications; D007249:Inflammation / Q000150:complications; D009206:Myocardium / Q000473:pathology*; D011379:Prognosis; D013318:Stroke Volume / Q000502:physiology; D018497:Ventricular Dysfunction, Right / Q000000981:diagnostic imaging* / Q000503:physiopathology
keywords: Cystic fibrosis; Heart