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The cardiomyopathy of cystic fibrosis: a modern form of Keshan disease
Javier Segovia-Cubero1,2, Lorena Ruiz-Bautista1, Luis Maiz-Carro3
1Cardiolology Dept., Hospital Universitario Puerta de Hierro, Madrid, Spain.
Frontiers in Cardiovascular Medicine
|February 16, 2024
Summary
Approximately 10% of adults with cystic fibrosis (CF) exhibit cardiac dysfunction, presenting features similar to Keshan disease. Selenium deficiency is linked to this left ventricular dysfunction in CF patients.
Area of Science:
- Cardiology
- Pulmonology
- Nutritional Science
Background:
- Cystic Fibrosis (CF) is associated with various complications, but cardiac involvement is not well-characterized.
- A specific cardiomyopathy in CF patients has not been previously described.
Purpose of the Study:
- To determine the prevalence of structural heart disease in CF patients.
- To characterize a novel cardiomyopathy in this population.
- To investigate the link between cardiac issues and nutritional deficiencies in CF.
Main Methods:
- Study included 3 cardiomyopathy (CMP) CF patients and 120 adult CF patients.
- Methods involved clinical exams, blood tests (vitamins, trace elements), echocardiography with myocardial strain analysis.
- Cardiac magnetic resonance imaging (CMR) and histopathological studies were performed.
Main Results:
- 10% prevalence of left ventricular (LV) dysfunction in the prospective CF cohort.
- Altered myocardial strain parameters observed even in CF patients with normal-appearing hearts.
- Histopathology revealed multifocal myocardial fibrosis resembling Keshan disease; CMR showed characteristic late gadolinium uptake.
- Selenium deficiency correlated with worse LV dysfunction, higher NTproBNP, and more severe CF complications.
Conclusions:
- Significant cardiac involvement, resembling Keshan disease, affects 10% of adult CF patients.
- Selenium deficiency is associated with the presence and severity of LV dysfunction in CF.
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