An observational study in Cystic Fibrosis, Cystic Fibrosis-related Diabetes and Cystic Fibrosis, Pulmonary, sponsored by Herlev and Gentofte Hospital. Active, not recruiting at 2 sites in Denmark. Open to participants aged 18 Years and older. Per ClinicalTrials.gov, last updated 2025-03-13.
Sponsored by Herlev and Gentofte Hospital · Observational
In a prospective observational cohort study (n = 100), the investigators aim to assess the correlation between cardiac biomarkers, advanced echocardiography and cystic fibrosis genotype and severity and determine whether these are prognostic markers of heart disease in patients suffering from cystic fibrosis (CF).
Background:
Previous studies using standard echocardiography have shown contradictory results regarding left ventricle (LV) structure and function in patients with CF. By the use of strain rate analyses, few studies have identified LV systolic abnormalities even in the setting of preserved conventional echocardiographic measures of LV systolic function such as left ventricular ejection fraction (LVEF) and fractional shortening. The expression of the cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel in myocytes of different mammalian species including mice and humans has been known for many years. Two studies showed that CFTR is involved in regulation of cardiomyocyte contraction in mice and another study demonstrated that loss of CFTR function in mice led to LV remodeling and increased aortic stiffness. The presence of CFTR in the myocardium has prompted debate regarding a possible intrinsic cardiac impairment due to loss of CFTR. A recent study found an association between severity of genotype in patients with CF and cardiac impairment. Several studies of pediatric CF patients with none or mild respiratory manifestations have also demonstrated early signs of cardiovascular impairments, such as arterial stiffness, increased pulmonary artery pressure (PAP) and abnormalities of right ventricular (RV) structure and function.
Primary hypothesis: CF is independently associated with higher prevalence of asymptomatic cardiac dysfunction and abnormal cardiac structure determined by conventional and advanced echocardiographic deformation measures (cross sectional study).
Secondary hypotheses: 1) CF is associated with elevated levels of specific cardiac biomarkers (cross sectional study). 2) CF is associated with higher pulmonary artery systolic pressure and higher prevalence of pulmonary hypertension determined by echocardiography (cross sectional study). 3) Asymptomatic cardiac dysfunction and abnormal cardiac structure assessed by conventional and advanced echocardiographic deformation measurements are early markers identifying CF patients in high risk of cardiac disease (prospective study).
Objective:
In a prospective observational cohort study (n = 100) the aim is to investigate the correlation between cardiac biomarkers, advanced echocardiography and cystic fibrosis (CF) genotype and severity and determine whether these are prognostic markers of heart disease in patients suffering from CF.
Design and control group:
The study is a prospective observational cohort study consisting of a random sample of consecutive patients from a population of outpatients with CF and a control group from the general population. The project group aims to include 100 participants with CF. The findings in CF participants will be compared with findings in the general population to estimate the risk of cardiovascular disease in CF patients. The investigators expect an inclusion period of approximately 6 months with register-based follow-up after 2, 5 and 10 years after inclusion.
The control group will consist of a random sample of age- and sex-matched patients (n=100) from the general population examined in the5th Copenhagen City Heart Study, 2011-2015 (ClinicalTrials.gov identifier NCT02993172, I-Suite no. 03741, National Committee on Health Research Ethics approval HEH-2015-045).
Baseline cardiovascular examination:
Baseline examination at CF Centre Copenhagen:
All included participants will undergo a physical examination by a physician at the CF center as well as an experienced pulmonary physiotherapist. The examinations will be performed at inclusion and prior to echocardiographic examination and contain the following: 1) Clinical assessment, 2) Pulmonary function testing.
Moreover, data on genetics and health status will be retrieved from the medical records and the national CF database. Diagnoses and/or medical history obtained will include:
CF genotype CF mutation type and mutation class CF-related comorbidities: CF-related diabetes, pancreatic insufficiency, liver steatosis/fibrosis, pulmonary function including high resolution CT scans, kidney disease, infectious disease burden and chronic infections as well as use of acute and chronic antibiotics.
Cardiovascular comorbidities: Stroke, peripheral artery disease (PAD), atrial fibrillation/atrial flutter and/or other cardiac arrythmias, pacemaker, kidney disease, hypertension, hypercholesterolemia, valvular disease (mitral, aortic, tricuspid and pulmonic valve disease), previous heart surgery, ischemic heart disease including non-invasive ischemic imaging results, prior myocardial infarction, prior revascularization and/or coronary artery bypass graft (CABG), heart failure, sleep apnea, venous thromboembolic syndrome (VTE) (deep vein thrombosis, pulmonary embolism).
Data management and statistics:
The General Data Protection Regulation and the Data Protection Act will be complied with.
All data will be stored in a password-protected electronic research database, REDCap, The Capital Region of Denmark's electronic data system. Questionnaires, signed consent forms and other sensitive documents will be kept in a locked archive in a locked office at the Department of Cardiology. The data management plan has been approved by the Danish Data Protection Agency (P-2022-366), and all data will be handled confidentially according to Danish law.
Baseline characteristics across the endpoints will be compared with trend tests using linear regression for continuous Gaussian distributed variables, by an extension of the Wilcoxon rank-sum test for continuous Gaussian distributed variables and by chi-square test for trend for proportions. Rates of all events will be calculated as the number of events divided by person-time at risk and stratified according to the primary endpoints. Hazard ratios (HR) will be calculated by Cox proportional hazards regression analysis. C-statistics will be obtained from univariable Cox models. Non-Gaussian distributed continuous variables will be categorized as dichotomous variables. The assumptions of proportional hazards in the models will be tested based on residuals. Predictive models for predicting the risk of future heart disease will be constructed using logistic regression. A p-value \<= 0.05 in 2-sided test will be considered statistically significant.
1,619 studies on the registry are indexed under Cystic Fibrosis; 199 are open to participants now.
This study's enrollment of 104 is above the median of 85 across 503 observational studies indexed under Cystic Fibrosis.
Browse Cystic Fibrosis studies →Herlev and Gentofte Hospital is the lead sponsor of 75 studies on the registry; 25 are open to participants now.
Counted across the registry records on this site, refreshed daily.
Adult CF patients (≥18 years) at Department of Infectious Diseases, CF Centre, Rigshospitalet, will be invited consecutively to participate in this study regardless of health status. Potential participants are invited by their regular CF physician to participate during routine visits at the CF Centre outpatient clinic.
Go through the criteria one by one — yes, no or not sure — and take the list to your doctor or the study team. Kept in this browser only.
Exclusion Criteria:
Underlined terms in the criteria open these too. Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
Before you join, the study team explains what taking part involves — its purpose, what happens, the risks and possible benefits — and answers your questions, so you can decide freely. Consent is usually recorded on a form (the informed consent form, ICF) signed by you or, where the law allows, by someone legally authorised to decide for you.
You can keep asking questions during the study, you are told of new information that matters, and you can choose to leave at any time.
Informed consent
Before you join, the study team explains what taking part involves — its purpose, what happens, the risks and possible benefits — and answers your questions, so you can decide freely. Consent is usually recorded on a form (the informed consent form, ICF) signed by you or, where the law allows, by someone legally authorised to decide for you.
You can keep asking questions during the study, you are told of new information that matters, and you can choose to leave at any time.
Explained by clinicaltrials.gg, not part of the registry record. The study team decides who can take part.
Adult CF patients (\>/=18 years) at Department of Infectious Diseases, Copenhagen University Hospital, will be invited to participate in this study. Potential participants are invited by their regular CF physician to participate during routine visits at the CF outpatient clinic. Inclusion criteria: Diagnosis of CF and age \>/=18 years. Exclusion criteria: * Lung transplanted patients * Inability to cooperate * Inability to understand and sign informed consent
The control group will consist of a random sample of age- and sex-matched participants from the general population examined in the 5th Copenhagen City Heart Study, 2011-2014 (ClinicalTrials.gov identifier NCT02993172, I-Suite no. 03741, National Committee on Health Research Ethics approval HEH-2015-045). Existing data from the Copenhagen City Heart Study will be transferred to the current study and will include personal identification number from the Central Office of Civil Registration, echocardiographic assessments, electrocardiograms as well as health related data (health conditions including symptoms, risk factors for cardiovascular disease, medication, prior clinical and/or paraclinical assessments including blood test results and procedures relevant to psoriasis and potential heart disease).
Rate of cardiovascular mortality
Time frame: 2, 5 and 10 year follow-up
Incidence of myocardial infarction
Time frame: 2, 5 and 10 year follow-up
Incidence of coronary revascularization (percutaneous coronary intervention/coronary artery bypass graft)
Time frame: 2, 5 and 10 year follow-up
Incidence of heart failure
Time frame: 2, 5 and 10 year follow-up
Rate of all-cause mortality
Time frame: 2, 5 and 10 year follow-up
Incidence of stroke
Time frame: 2, 5 and 10 year follow-up
Number of Participants with admission with cardiac heart failure
Time frame: 2, 5 and 10 year follow-up
Number of Participants with admission with stroke
Time frame: 2, 5 and 10 year follow-up
Plan to share: Undecided
Ran from Jan 2023 to primary completion in Mar 2023, 2 months.
Of 228 non-industry observational studies under Cystic Fibrosis, half reached primary completion within 40 months of starting (the median), counting those still running; a quarter within 19 and three quarters within 89. This study's 2 months is below that.
Counting every sponsor, half of 243 studies reached it within 41 months; a quarter within 19 and three quarters within 79.
How these figures are worked out, and which studies are left out of them.
This study is active, not recruiting, as verified in Nov 2024. You cannot join it, but the record below documents what was studied.
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Herlev and Gentofte Hospital