Clinical trial NCT04737317: Diagnostic Criteria in Cardiac Sarcoidosis
Official title: Evaluation of Diagnostic Criteria in Cardiac Sarcoidosis - an Observational Study
Study type: OBSERVATIONAL
Overall status: RECRUITING
Conditions: Cardiac Sarcoidosis
Lead sponsor: Heart Center Leipzig - University Hospital
Sponsor class: OTHER
First posted: 2021-02-03
Enrollment: 100
Sex: ALL
Minimum age (years): 18
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: PROSPECTIVE
Sampling method: PROBABILITY_SAMPLE
Study population: Patients presenting at the Heart Center Leipzig, elective and urgent admissions

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.

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

Primary outcomes:
measure: Clinical recovery with immunosuppressive therapy
timeFrame: 1 year
measure: Imaging recovery with immunosuppressive therapy
timeFrame: 1 year
Secondary outcomes:
measure: Cardiac mortality
timeFrame: 2 years
measure: All-cause mortality
timeFrame: 2 years
measure: Unplanned hospitalizations
timeFrame: 2 years
measure: Change in LV-EF from baseline
timeFrame: 2 years
measure: Change in RV function
timeFrame: 2 years
measure: Patients' quality of life
timeFrame: 2 years
measure: VT ablation success
timeFrame: 2 years
measure: Adverse events of immunosuppressive therapy
timeFrame: 2 years
measure: Changes of immunosuppressive therapy due to lack of success
timeFrame: 2 years
Other outcomes:
Locations:
city: Leipzig
country: Germany
facility: Heart Center of Leipzig
Study officials:
affiliation: Heart Center Leipzig - University Hospital, Department Electrophysiology
name: Borislav Dinov, MD
role: PRINCIPAL_INVESTIGATOR
affiliation: Heart Center Leipzig - University Hospital, Department Electrophysiology
name: Laura Ueberham, MD
role: STUDY_CHAIR
References: