Diagnostic Criteria in Cardiac Sarcoidosis
For patients and families
In plain language
An automatic summary of structured registry data. It is an orientation aid, not a substitute for the official protocol or a physician assessment.
- What is being studied
- The protocol lists: ECG, Echo, laboratory tests, CMR, PET-CT, Biopsy.
- Who it may be relevant to
- Registry conditions: Cardiac Sarcoidosis. Basic parameters: from 18 years · All.
- What needs checking
- Age, condition and sex are only basic indicators. Prior treatment, laboratory values and other mandatory requirements appear in the eligibility criteria below.
- Where it takes place
- Germany
- Next step
- Save the trial, show it to the treating physician, and confirm current recruitment with the study center. Costs, documents and travel →
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Official title
Evaluation of Diagnostic Criteria in Cardiac Sarcoidosis - an Observational Study
Overview
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.
Interventions
- Diagnostic test 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
Primary outcome measures
- Clinical recovery with immunosuppressive therapy [Time frame: 1 year]
- Imaging recovery with immunosuppressive therapy [Time frame: 1 year]
Secondary outcome measures (9)
- Cardiac mortality [Time frame: 2 years]
- All-cause mortality [Time frame: 2 years]
- Unplanned hospitalizations [Time frame: 2 years]
- Change in LV-EF from baseline [Time frame: 2 years]
- Change in RV function [Time frame: 2 years]
- Patients' quality of life [Time frame: 2 years]
- VT ablation success [Time frame: 2 years]
- Adverse events of immunosuppressive therapy [Time frame: 2 years]
- Changes of immunosuppressive therapy due to lack of success [Time frame: 2 years]
Eligibility criteria
Inclusion criteria
- patients with suspected CS due to the following presentations:
- age < 65 years and new higher degree AV block, causative coronary artery disease excluded
- age < 65 years and new ventricular tachycardia, causative coronary artery disease excluded
- age < 65 years and ventricular tachycardia, causative coronary artery disease excluded
- 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
Criteria are shown verbatim from the registry (in English). Final eligibility is always assessed by the study center.
Healthy volunteers: No
Study design
- Observational model
- Cohort
Study locations
Germany · 1 center
- Heart Center of Leipzig — Leipzig
Identifiers
NCT: NCT04737317 · 20201231PV1