Abstract
Background
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.
Methods
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.
Results
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).
Conclusions
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
Funding Acknowledgement
Type of funding source: None
Angiotensin converting enzyme inhibitor therapy promotes remodeling in patients with left ventricular ejection fraction ⪢ 40% after acute transmural myocardial infarction
