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Cardiac sarcoidosis: frequency, diagnostic approach and follow-up in a tertiary center

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Cardiac sarcoidosis (CS) is associated with poor prognosis, making early diagnosis and treatment important. The aim of this study is to evaluate our diagnostic results and follow-up for the diagnosis… Click to show full abstract

Cardiac sarcoidosis (CS) is associated with poor prognosis, making early diagnosis and treatment important. The aim of this study is to evaluate our diagnostic results and follow-up for the diagnosis of CS in a tertiary center. We studied 188 patients with proven extra-cardiac sarcoidosis referred to our outpatient clinic for evaluation of cardiac involvement. Eight patients were excluded because electrocardiogram (ECG) and/or transthoracic echocardiography (TTE) was missing. Cardiac magnetic resonance (CMR) and/or positron emission tomography (PET) was performed in 66% and 37% of the patients, respectively. Median follow-up duration was 2.9 [1.2–5.3] years. The diagnosis of CS was based on the Heart Rhythm Society criteria. Cardiac symptoms defined as palpitations, angina, dyspnea and (near)-syncope were present in 156 of 180 (87%) patients. Any abnormality on ECG (bundle branch blocks, atrioventricular blocks, sinus tachycardia or atrial fibrillation) and/or TTE (left ventricular ejection fraction <55%, presence of regional wall abnormalities or myocardial hypertrophy) was found in 92/180 (51%) patients. CS was diagnosed in 42 of 180 (23%) patients, of whom 31 (74%) had any ECG and/or TTE abnormalities. However, ECG and/or TTE abnormalities were also present in 44% of the patients without cardiac involvement. Patients with CS showed a second type II or third degree AV-blocks in 3/42 (7%), a left ventricular ejection fraction <35% on TTE in 9/42 (21%), late gadolinium enhancement by CMR consistent with CS in 28/34 (82%), and myocardial FDG uptake by PET in 19/31 (61%). In 84 of the 138 patients without cardiac involvement, CMR was performed. In 15 patients an alternative diagnosis was found (i.e. myocardial infarction or other non-ischemic cardiomyopathy). The estimated 8-year cumulative event rate composite endpoint of sustained ventricular tachycardia, ventricular fibrillation, aborted sudden cardiac death, heart transplantation and all-cause mortality was 41% in the CS patients and 12% in the patients without CS (Figure 1, p<0.001). In our study, 23% of the patients with proven extra-cardiac sarcoidosis was diagnosed with CS. Cardiac symptoms, ECG and TTE were of limited diagnostic value for screening for CS. CMR provided a good diagnostic yield and identified other cardiac diseases in a substantial number of patients. Figure 1. KMCurve_CompositeEndpoint Type of funding source: None

Keywords: diagnosis; sarcoidosis; tte; cardiac sarcoidosis; tertiary center

Journal Title: European Heart Journal
Year Published: 2020

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