Cardiac Structure and Function in People with Cystic Fibrosis

Area of Science:

• Cardiology
• Pulmonology
• Genetics

Background:

• Cystic fibrosis (CF) is a genetic disorder with increasing life expectancy due to advanced treatments.
• Cardiac involvement in CF is not fully understood, necessitating further investigation.
• New cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies warrant updated research on CF-related cardiac conditions.

Purpose of the Study:

• To determine the prevalence of cardiac alterations in people with CF (pwCF) compared to matched controls.
• To identify potential cardiovascular risk factors associated with cardiac dysfunction in pwCF.
• To evaluate the impact of CFTR modulator treatment on cardiac function.

Main Methods:

• A cross-sectional study involving 104 pwCF and 104 matched controls.
• Clinical and echocardiographic assessments were performed on all participants.
• Multivariable analysis was used to adjust for potential confounding factors.

Main Results:

• 44% of pwCF exhibited abnormal cardiac function, more than double the rate in controls.
• pwCF demonstrated smaller left ventricular dimensions, impaired left ventricular diastolic function, and reduced right and left ventricular systolic function.
• Male sex and a decreasing FEV1/FVC ratio were independently associated with abnormal cardiac function in pwCF.

Conclusions:

• Significant left- and right-sided cardiac alterations are present in pwCF.
• Impaired RV and LV systolic function persist in pwCF after adjusting for risk factors.
• Male sex and reduced pulmonary function (FEV1/FVC ratio) are associated with abnormal cardiac function in individuals with CF.