Cardiac Arrhythmia, Cardiac Diseases, Congestive Heart Failure, Heart Block, Systemic Sclerosis
Conditions
Brief summary
Systemic sclerosis is an orphan, multiorgan disease affecting the connective tissue of the skin and all internal organs. Cardiac involvement, mainly characterised by small intramyocardial coronary artery involvement and myocardial fibrosis, can cause the development of impaired diastolic ventricular filling, cardiac blocks and ventricular arrhythmias, and can ensue in congestive heart failure and sudden death. Until now, no drug has been proven to have a therapeutic effect on SSc myocardial disease on an evidence-based level. Short-term trials and retrospective studies have suggested a favourable and protective effect of calcium channel blockers and angiotensin converting enzyme inhibitors in patients with myocardial involvement. However, no data are presently available on the prevention and treatment of severe heart disease. This observational trial is part of the collaborative project DeSScipher, one out of five observational trials to decipher the optimal management of systemic sclerosis. Aim of this observational trial is to assess the efficacy and safety of calcium channel blockers and angiotensin converting enzyme inhibitors in asymptomatic SSc patients with cardiac involvement.
Interventions
None listed
Sponsors
European Union
University of Giessen
University of Zurich
University of Paris 5 - Rene Descartes
University of Florence
University of Basel
University College, London
Charite University, Berlin, Germany
University of Pecs
University of Leeds
Schoen Klinik Hamburg Eilbek
Gabriele Valentini
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Healthy volunteers
No
Inclusion criteria
* Juvenile and adult systemic sclerosis patients, with diagnosis according to the SSc ACR/EULAR criteria or the PRES/ACR/EULAR juvenile SSc criteria respectively * Asymptomatic (for cardiac disease) systemic sclerosis patients at risk for severe heart disease with at least one of the following risk factors: male sex and/or DLCO lower than 80% and/or sPAP \> 30 mmHg and/or synovitis and/or joint contractures and/or digital ulcers and/or proteinuria. Asymptomatic for cardiac disease is defined by patients without dyspnea NYHA \>/= II, without palpitations and without bilateral leg edema.
Exclusion criteria
* Any significant pulmonary parenchymal (FVC \< 70% and/or DLCO \< 70%), pulmonary vascular (estimated systolic PAP \> 40 mmHg), gastrointestinal (malabsorption syndrome or paralytic ileus) or renal (serum creatinine level \>1.2 mg/dl, dialysis or previous scleroderma renal crisis) involvement * Patients with dyspnea class NYHA \>/= II * Patients with palpitations * Patients with bilateral leg edema.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Cumulative incidence of CB, VA, pacemaker implantation, congestive heart failure and sudden death | 1 years | Cumulative incidence of cardiac blocks, ventricular arrhythmias, pacemaker implantation, congestive heart failure and sudden death. |
Other
| Measure | Time frame |
|---|---|
| Incidence of drug-related adverse events, incidence of withdrawal from treatment due to drug-related adverse events | 1 year |
Countries
France, Germany, Hungary, Italy, Switzerland, United Kingdom
Contacts
Primary ContactGabriele Valentini, Prof.
Outcome results
None listed