Abstract

BackgroundSarcoidosis is a systemic granulomatous disease that may affect the myocardium. This study evaluated the diagnostic and prognostic value of 2-dimensional speckle tracking echocardiography in cardiac sarcoidosis (CS).MethodsEighty-three patients with extracardiac, biopsy-proven sarcoidosis and definite/probable diagnosis of cardiac involvement diagnosed from January 2005 through December 2016 were included. Strain parameters in early stages of CS, in a subgroup of 23 CS patients with left ventricular ejection fraction (LVEF) within normal limits (LVEF> 52% for men: > 54% for women, mean value: 57.3% ± 3.8%) and no wall motion abnormalities was compared with 97 controls (1:4) without cardiac disease. LV and right ventricular (RV) global longitudinal (GLS), circumferential (GCS), and radial (GRS) strain and strain rate (SR) analyses were performed with TomTec software and correlated with cardiac outcomes (including heart failure and arrhythmias). This study was approved by the Mayo Clinic Institutional Review Board, and all patients gave informed written consent to participate.ResultsMean age of CS patients was 53.6 ± 10.8 years, and 34.9% were women. Mean LVEF was 43.2% ± 12.4%; LV GLS, − 12.4% ± 3.7%; LV GCS, − 17.1% ± 6.5%; LV GRS, 29.3% ± 12.8%; and RV wall GLS, 14.6% ± 6.3%. In the 23 patients with early stage CS with normal LVEF and RV systolic function, strain parameters were significantly reduced when compared with controls (respectively: LV GLS, − 15.9% ± 2.5% vs − 18.2% ± 2.7% [P = .001]; RV GLS, − 16.9% ± 4.5% vs − 24.1% ± 4.0% [P < .001]). A LV GLS value of − 16.3% provided 82.2% sensitivity and 81.2% specificity for the diagnosis of CS (AUC 0.91), while a RV value of − 19.9% provided 88.1% sensitivity and 86.7% specificity (AUC 0.93). Hospital admission and heart failure significantly correlated to impaired LV GLS (> − 14%).ConclusionReduced strain values in the LV GLS and RV GLS can be used in the diagnostic algorithm in patients with suspicion of cardiac sarcoidosis. These values also correlate with adverse cardiovascular events.

Highlights

  • Sarcoidosis is a systemic granulomatous disease that may affect the myocardium

  • We retrospectively identified 122 patients with systemic sarcoidosis that were evaluated at our Department of Cardiovascular Medicine and had a transthoracic echocardiogram performed for suspected cardiac sarcoidosis (CS) from January 1, 2005 through December 31, 2016

  • Among the 83 patients who met the histologic and clinical criteria of CS according to the guidelines of the Heart Rhythm Society (HRS) consensus statement, [12] 25 patients had a definite CS diagnosis and 58 a probable CS diagnosis (24 with perfusion defects and FDG uptake on FDG-PETand 34 patients with positive cardiac magnetic resonance (CMR) findings, in which 16 had an inflammatory pattern with increased signal intensity T2 sequences and 18 had patchy delayed enhancement with non-coronary distribution)

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Summary

Introduction

Sarcoidosis is a systemic granulomatous disease that may affect the myocardium. This study evaluated the diagnostic and prognostic value of 2-dimensional speckle tracking echocardiography in cardiac sarcoidosis (CS). Myocardial granulomas are detected post-mortem in 12 to 25% of patients with extracardiac sarcoidosis [1, 2]. These granulomas lead to subclinical myocardial inflammation, edema, fibrosis, and scars, which in turn result in ventricular remodeling and systolic dysfunction [3]. Conventional echocardiography may identify typical myocardial alterations due to sarcoidosis, such as wall aneurysms, wall motion abnormalities, and thinning of the basal septum, but it may underestimate regional myocardial dysfunction since in the early phases of the disease it can appear normal [10]. Cardiac magnetic resonance (CMR) and fluorodeoxyglucose–positron emission tomography (FDG–PET) imaging may be useful to detect edema, active inflammation or scars in myocardial walls [11] but they are not always available

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