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

Fabien Labombarda1, Eric Saloux1, Jacques Brouard2

  • 1Caen CHU, Department of Cardiology, Caen, F-14000, France.

Respiratory Medicine
|September 1, 2016
PubMed

Insights

Cystic fibrosis (CF) can affect the heart, causing dysfunction. Echocardiography reveals this cystic fibrosis-related cardiomyopathy, influenced by hypoxemia and inflammation, impacting patient risk stratification.

Area of Science:

  • Cardiology
  • Pulmonology
  • Genetics

Background:

  • Cystic fibrosis is a multi-systemic disease with progressive organ dysfunction.
  • Evidence suggests myocardial involvement in cystic fibrosis, termed cystic fibrosis-related cardiomyopathy.
  • Hypoxemia, inflammation, and CFTR dysfunction are implicated in cardiac issues.

Purpose of the Study:

  • To review evidence for and against heart involvement in cystic fibrosis.
  • To explore the mechanisms and prognostic implications of cystic fibrosis-related cardiomyopathy.
  • To synthesize findings from echocardiographic studies on cardiac function in CF.

Main Methods:

  • Literature review focusing on echocardiographic studies.
  • Analysis of pathological, experimental, and clinical evidence.
  • Synthesis of data on ventricular dysfunction in cystic fibrosis patients.

Main Results:

  • Echocardiography demonstrates diastolic and/or systolic ventricular dysfunction in cystic fibrosis.
  • Hypoxemia and inflammation are key factors in cardiac involvement.
  • Cystic Fibrosis Transmembrane Regulator (CFTR) plays a role in cardiomyocyte function.

Conclusions:

  • Cystic fibrosis-related cardiomyopathy is a recognized entity.
  • Cardiac function assessment aids in risk stratification and treatment decisions for CF patients.
  • Further investigation into heart involvement in CF is warranted.

Keywords:
Cystic fibrosisHeart

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