Heart involvement in cystic fibrosis: A specific cystic fibrosis-related myocardial changes?

Respir Med. 2016 Sep;118:31-38. doi: 10.1016/j.rmed.2016.07.011. Epub 2016 Jul 15.

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.

Keywords: Cystic fibrosis; Heart.

Publication types

  • Review

MeSH terms

  • Cardiomyopathies / complications
  • Cardiomyopathies / physiopathology
  • Cystic Fibrosis / complications*
  • Cystic Fibrosis / diagnostic imaging*
  • Cystic Fibrosis / pathology
  • Cystic Fibrosis / physiopathology
  • Echocardiography / methods
  • Heart Ventricles / diagnostic imaging*
  • Heart Ventricles / physiopathology
  • Humans
  • Hypoxia / complications
  • Inflammation / complications
  • Myocardium / pathology*
  • Prognosis
  • Stroke Volume / physiology
  • Ventricular Dysfunction, Right / diagnostic imaging*
  • Ventricular Dysfunction, Right / physiopathology