Diagnostic Criteria in Cardiac Sarcoidosis

NCT ID: NCT04737317

Last Updated: 2023-05-09

Basic Information

• Recruitment Status: RECRUITING
• Total Enrollment: 100 participants
• Study Classification: OBSERVATIONAL
• Study Start Date: 2023-05-01
• Study Completion Date: 2027-01-01

Brief Summary

The purpose of the study is to evaluate the accuracy of the current diagnostic criteria of cardiac sarcoidosis.

Detailed Description

Sarcoidosis is an inflammatory condition rarely affecting the heart, exhibiting fluctuating disease activity and eventually leading to ventricular fibrosis. Clinical presentation ranges from no symptoms at all to life-threatening arrhythmias, heart failure and death in severe cases. The heart lesions show patchy distribution and can mimic virtually any other type of cardiomyopathy, which poses significant challenges for the accurate and eary diagnosis.

At present, there are four diagnostic tools and recomendations for diagnosis of cardiac sarcoidosis (CS) that utilize clinical, ECG, laboratory, imaging and biopsy criteria. Recently, it was demonstrated that there is a significant diagreement between these four diagnostic tools. On the other hand, earlier identification of patients with active inflammatory process is necessary, as they are those who will most likely benefit from immunosuppressive therapy.

The aim of this observational study is to evaluate the prognostic significance of the current clinical, ECG, laboratory, imaging and biopsy criteria for diagnosing cardiac sarcoidosis. Patients with suspected cardiac sarcoidosis based on clinical symptoms will recieve elaborate diagnostic work-up consisting of: evaluation of symptoms, family history, occupational hazards, ECG, chest X-ray, laboratory markers for sarcoidosis, positron emission tomography (PET), cardiac magnetic resonance (CMR) as well as endomyocardial biopsy.

In accordance to the recomendations of the current guidelines, the patients will be devided into 3 groups: 1) patients with proven CS; 2) patients with probable CS; and 3) unlikely CS that will serve as a reference group. The patients in first two groups will be treated with immunosuppressive therapy, mainly glucocorticoids (GC). The follow-up at 3, 6, 12 and 24 months will consist of evaluation of the symptoms using dedicated King's Sarcoidosis Questionaire (KSQ), fatigue assessment scale (FAS), Holter-ECG, pacemaker interrogation, laboratory findings, imaging with PET and CMR.

Clinical outcomes will be defined as: 1) improvement of symptoms (FAS score, heart failure class, AV block, ventricular arrhythmias, and KSQ score) and 2) improvement of imaging parameters ( left ventricular ejection fraction, PET activity, myocardial edema, scar in CMR). The outcomes between the groups will be compared using group 3 as reference. Furthermore, patients with improvement with GC (successful therapy) will be compared with those without improvement (unsuccessful therapy) and the predictive value of each diagnostic criterion will be evaluated. Finally, the odds ratios (OR) for each parameter will be used to calculate a diagnostic and predictive score which will be used prospectively to evaluate patients with suspected CS and to guide the therapy.

Conditions

Cardiac Sarcoidosis

Study Design

• Observational Model Type: COHORT
• Study Time Perspective: PROSPECTIVE

Interventions

ECG, Echo, laboratory tests, CMR, PET-CT, Biopsy

ECG - 12 leads ECG Echo: transthoracic echocardiography laboratory tests: full blood count, electrolytes, creatinine, high sensitive cardiac troponin T, angiontesin converting enzyme, soluble interleukin-2 receptor, cardiac magnetic resonance imaging: 1.5 Tesla scanner, Conventional cine imaging and late gadolinium enhancement imaging PET-CT: 18F-fluorodeoxyglucose (FDG)- positron emission tomograph Biopsy: cardiac biopsy = endomyocardial biopsy, left and right ventricle, at least 5 specimens in formaldehyd and 3 specimens for testing of infectious agents Biopsy: extracardiac biopsy, as accessible according to imaging

Intervention Type: DIAGNOSTIC_TEST

Eligibility Criteria

Inclusion Criteria

• patients with suspected CS due to the following presentations:

1. age < 65 years and new higher degree AV block, causative coronary artery disease excluded

2. age < 65 years and new ventricular tachycardia, causative coronary artery disease excluded

3. age < 65 years and ventricular tachycardia, causative coronary artery disease excluded

4. extracardiac sarcoidosis and cardiac involvement suggested (palpitations, abnormal ECG, abnormal echocardiography)

To diagnose CS one of the following diagnostic tools will be used:

A) World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG)

B) Heart Rhythm Society expert consensus statement on the diagnosis and management of arrhythmias associated with cardiac sarcoidosis

C) Japanese Society of Nuclear Cardiology (JSNC)

Patients whit histological confirmation from myocardial tissue fullfil will be defined as proven CS. (Group 1)

Patients with clinical and imaging findings highly suggesting CS, but without histological confirmation from myocardial biopsy will be defined as probable CS (Group 2)

Patients who do not fullfil the criteria or exhibiting findings suggesting an alternative, more likely diagnosis, will be considered as unlikely CS (Group 3)

Exclusion Criteria

• unable or unwilling to provide informed consent
• patients who are pregnant or lactating
• noncompliant patients refusing the recommended therapy
• age < 18 years

• Minimum Eligible Age: 18 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

Heart Center Leipzig - University Hospital

OTHER

Sponsor Role: lead

Responsible Party

Borislav Dinov

Head of Ventricular Tachycardia Unit

Responsibility Role: PRINCIPAL_INVESTIGATOR

Principal Investigators

Borislav Dinov, MD

Role: PRINCIPAL_INVESTIGATOR

Heart Center Leipzig - University Hospital, Department Electrophysiology

Laura Ueberham, MD

Role: STUDY_CHAIR

Heart Center Leipzig - University Hospital, Department Electrophysiology

Locations

Heart Center of Leipzig

Leipzig, , Germany

Site Status: RECRUITING

Countries

Germany

Central Contacts

Borislav Dinov, MD

Role: CONTACT

borislav.dinov@helios-gesundheit.de

+49 341 865 1431

Laura Ueberham, MD

Role: CONTACT

laura.ueberham@helios-gesundheit.de

+49341 865 1431

Facility Contacts

Borislav Dinov, PhD

Role: primary

Borislav.Dinov@helios-gesundheit.de

+49341865252134

Hans Ebbinghaus, MD

Role: backup

Hans.Ebbinghaus@helios-gesundheit.de

+49341865252550

Other Identifiers

20201231PV1

Identifier Type: -

Identifier Source: org_study_id