Clinical Investigation
Myocardial Disorders
Myocardial Strain Assessment in Cystic Fibrosis

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Background

The aim of this work was to evaluate myocardial strain analysis as a tool for the early detection of left ventricular functional changes in patients with cystic fibrosis.

Methods

A total of 42 consecutive patients (mean age, 24 ± 7.5 years; 52% men) diagnosed with cystic fibrosis and referred for echocardiographic cardiac function assessment were prospectively enrolled. A group of healthy age-matched and gender-matched volunteers (n = 42) formed the reference population for echocardiographic comparisons.

Results

Left ventricular ejection fraction was conserved in both groups but was significantly lower in the cystic fibrosis group. Cardiac function assessment using Doppler tissue imaging parameters revealed that both systolic and diastolic measurements differed between the two groups: mitral peak systolic and diastolic velocities, as well as septal and lateral wall strain rates, were decreased in patients with cystic fibrosis, as was longitudinal strain of both the septal and lateral walls.

Conclusions

Using strain measurements, subclinical changes in left ventricular function were found in patients with cystic fibrosis. These parameters were correlated with the degree of pulmonary involvement severity. These findings have potentially significant clinical implications for the outcomes and follow-up of patients with cystic fibrosis, meriting further studies.

Section snippets

Study Population

We prospectively recruited patients with cystic fibrosis followed up at the Cystic Fibrosis Center of Basse-Normandie. Diagnoses had been confirmed using sweat chloride tests or molecular biology. To qualify for inclusion in the study, patients were required to be ≥16 years old, to be ambulatory and without signs of respiratory symptom worsening (no exacerbations requiring antibiotic therapy and no change in symptoms in the months before echocardiography), not to be on a lung transplantation

Patients

Overall, 42 consecutive patients (mean age, 24 ± 7.5 years; 52% men) diagnosed with cystic fibrosis and referred for echocardiographic assessment of cardiac function were enrolled in this prospective cohort. At the time of echocardiography, all patients were in stable respiratory condition and showed no signs of heart failure. Severe lung disease, defined as FEV1 < 30%, was present in 10 patients (23.8%), and oxygen supplementation was necessary in four patients (9.5%) with desaturation. The

Discussion

Using longitudinal strain measurements, we report for the first time to our knowledge an unexpectedly high rate of subclinical left ventricular functional changes in patients with cystic fibrosis and normal LVEFs.

Previous echocardiographic studies using standard two-dimensional, M-mode, and Doppler parameters have reported contradictory results concerning left ventricular systolic function in patients with cystic fibrosis.2 Our findings using longitudinal strain parameters suggest that left

Conclusions

Using longitudinal strain measurements, we found an unexpectedly high rate of subclinical left ventricular functional changes in patients with cystic fibrosis with normal LVEFs. These findings have potentially significant clinical implications for the outcomes and follow-up of patients with cystic fibrosis, meriting further studies.

Acknowledgments

We thank M. Hamon for helpful discussion. We thank Vaincre la Mucoviscidose (Paris, France) for support of French Cystic Fibrosis Centers.

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