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Peer-reviewedCC BY 4.0Journal of Clinical Medicine

Cardiovascular Manifestations of Cystic Fibrosis

  • Keshav Garg1
  • Ankit Agrawal2
  • Ahmad Mustafa1
  • Mohab Elnashar1
  • Gunner Gilbert3
  • Akiva Rosenzveig4
  • Arnav Lakkimsetti1
  • Elio Haroun5
  • Aro Daniela Arockiam6
  • Karim Hoyek4
  • Clement Tan4
  • Tom Kai Ming Wang4,7
7 affiliations
  1. 1Department of Internal Medicine, University of Arkansas for Medical Sciences, Little Rock, AR 72205, USA
  2. 2Department of Cardiovascular Medicine, University of Arkansas for Medical Sciences, Little Rock, AR 72205, USA
  3. 3Department of Internal Medicine, University of Arkansas for Medical Sciences, Fayetteville, AR 72703, USA
  4. 4Department of Cardiovascular Medicine, Heart, Vascular and Thoracic Institute, Cleveland Clinic, Cleveland, OH 44195, USA
  5. 5Department of Internal Medicine, Yale New-Haven Health System, New Haven, CT 06510, USA
  6. 6Department of Internal Medicine, Unity Health, Searcy, AR 72143, USA
  7. 7Green Lane Cardiovascular Service, Te Toka Tumai Auckland City Hospital, Auckland 1023, New Zealand
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Abstract

Cystic fibrosis (CF) has undergone a remarkable transformation from a fatal childhood disease to a chronic adult condition, with a median predicted survival now exceeding 50 years following the introduction of highly effective CFTR modulator therapy (HEMT). This demographic shift has unmasked cardiovascular complications that were previously unrecognized due to premature mortality. In a multinational propensity-matched cohort, CF conferred a 65% higher risk of major adverse cardiac events (MACEs), including myocardial infarction, heart failure, and atrial fibrillation, versus matched controls (Frost et al., HR 1.65). In a separate Canadian population-based cohort, cardiovascular disease occurred at 2.9 times the rate of the general population and at least 20 years earlier (Arya et al., RR 2.9). Cardiac involvement extends beyond the traditional paradigm of cor pulmonale. CFTR is expressed in cardiomyocytes, vascular endothelium, and smooth muscle, where its dysfunction disrupts ion homeostasis, impairs nitric oxide-dependent vasodilation, and promotes oxidative stress. Clinically, these mechanisms manifest as biventricular dysfunction detectable by strain echocardiography and cardiac MRI even when ejection fraction is preserved, diffuse myocardial fibrosis, endothelial dysfunction and accelerated arterial stiffness from childhood, increased arrhythmia susceptibility, and, in a single-center cohort, an unexpectedly high prevalence of pericardial calcification (18%) on chest CT that awaits independent replication. Paradoxically, while HEMT has dramatically improved pulmonary outcomes, it introduces new cardiovascular considerations including hypertension, dyslipidemia, and obesity, and cross-sectional data in modulator-treated cohorts show persistent cardiac abnormalities, though whether these predate treatment or would differ without it is unknown. This review synthesizes the genetic, molecular, and clinical dimensions of cardiovascular disease in CF; distinguishes direct CFTR-mediated cardiac effects from indirect consequences of chronic lung disease; examines vascular, electrophysiological, valvular, and pericardial manifestations; and discusses management implications including CFTR modulator therapy, exercise, nutritional paradigm shifts, and transplantation considerations.

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Cite

BibTeXRIS
BibTeX
@article{garg2026cardiovascular,
  title = {{Cardiovascular Manifestations of Cystic Fibrosis}},
  author = {Keshav Garg and Ankit Agrawal and Ahmad Mustafa and Mohab Elnashar and Gunner Gilbert and Akiva Rosenzveig and Arnav Lakkimsetti and Elio Haroun and Aro Daniela Arockiam and Karim Hoyek and Clement Tan and Tom Kai Ming Wang},
  journal = {Journal of Clinical Medicine},
  year = {2026},
  month = oct,
  doi = {10.3390/jcm15207988},
  url = {https://www.mdpi.com/2077-0383/15/20/7988},
  note = {CC BY 4.0},
}
RIS
TY  - JOUR
TI  - Cardiovascular Manifestations of Cystic Fibrosis
AU  - Keshav Garg
AU  - Ankit Agrawal
AU  - Ahmad Mustafa
AU  - Mohab Elnashar
AU  - Gunner Gilbert
AU  - Akiva Rosenzveig
AU  - Arnav Lakkimsetti
AU  - Elio Haroun
AU  - Aro Daniela Arockiam
AU  - Karim Hoyek
AU  - Clement Tan
AU  - Tom Kai Ming Wang
PY  - 2026
DA  - 2026/10/15
JO  - Journal of Clinical Medicine
DO  - 10.3390/jcm15207988
UR  - https://www.mdpi.com/2077-0383/15/20/7988
AB  - Cystic fibrosis (CF) has undergone a remarkable transformation from a fatal childhood disease to a chronic adult condition, with a median predicted survival now exceeding 50 years following the introduction of highly effective CFTR modulator therapy (HEMT). This demographic shift has unmasked cardiovascular complications that were previously unrecognized due to premature mortality. In a multinational propensity-matched cohort, CF conferred a 65% higher risk of major adverse cardiac events (MACEs), including myocardial infarction, heart failure, and atrial fibrillation, versus matched controls (Frost et al., HR 1.65). In a separate Canadian population-based cohort, cardiovascular disease occurred at 2.9 times the rate of the general population and at least 20 years earlier (Arya et al., RR 2.9). Cardiac involvement extends beyond the traditional paradigm of cor pulmonale. CFTR is expressed in cardiomyocytes, vascular endothelium, and smooth muscle, where its dysfunction disrupts ion homeostasis, impairs nitric oxide-dependent vasodilation, and promotes oxidative stress. Clinically, these mechanisms manifest as biventricular dysfunction detectable by strain echocardiography and cardiac MRI even when ejection fraction is preserved, diffuse myocardial fibrosis, endothelial dysfunction and accelerated arterial stiffness from childhood, increased arrhythmia susceptibility, and, in a single-center cohort, an unexpectedly high prevalence of pericardial calcification (18%) on chest CT that awaits independent replication. Paradoxically, while HEMT has dramatically improved pulmonary outcomes, it introduces new cardiovascular considerations including hypertension, dyslipidemia, and obesity, and cross-sectional data in modulator-treated cohorts show persistent cardiac abnormalities, though whether these predate treatment or would differ without it is unknown. This review synthesizes the genetic, molecular, and clinical dimensions of cardiovascular disease in CF; distinguishes direct CFTR-mediated cardiac effects from indirect consequences of chronic lung disease; examines vascular, electrophysiological, valvular, and pericardial manifestations; and discusses management implications including CFTR modulator therapy, exercise, nutritional paradigm shifts, and transplantation considerations.
SN  - 2077-0383
ER  -