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. 2016 Jan;9(1):e003738.
doi: 10.1161/CIRCIMAGING.115.003738.

Prognosis of Myocardial Damage in Sarcoidosis Patients With Preserved Left Ventricular Ejection Fraction: Risk Stratification Using Cardiovascular Magnetic Resonance

Gillian Murtagh  1 , Luke J Laffin  1 , John F Beshai  1 , Francesco Maffessanti  1 , Catherine A Bonham  1 , Amit V Patel  1 , Zoe Yu  1 , Karima Addetia  1 , Victor Mor-Avi  1 , Joshua D Moss  1 , D Kyle Hogarth  1 , Nadera J Sweiss  1 , Roberto M Lang  1 , Amit R Patel  2
Affiliations

Prognosis of Myocardial Damage in Sarcoidosis Patients With Preserved Left Ventricular Ejection Fraction: Risk Stratification Using Cardiovascular Magnetic Resonance

Gillian Murtagh et al. Circ Cardiovasc Imaging. 2016 Jan.

Abstract

Background: Cardiac sarcoidosis is associated with an increased risk of heart failure and sudden death, but its risk in patients with preserved left ventricular ejection fraction is unknown. Using cardiovascular magnetic resonance in patients with extracardiac sarcoidosis and preserved left ventricular ejection fraction, we sought to (1) determine the prevalence of cardiac sarcoidosis or associated myocardial damage, defined by the presence of late gadolinium enhancement (LGE), (2) quantify their risk of death/ventricular tachycardia (VT), and (3) identify imaging-based covariates that predict who is at greatest risk of death/VT.

Methods and results: Parameters of left and right ventricular function and LGE burden were measured in 205 patients with left ventricular ejection fraction >50% and extracardiac sarcoidosis who underwent cardiovascular magnetic resonance for LGE evaluation. The association between covariates and death/VT in the entire group and within the LGE+ group was determined using Cox proportional hazard models and time-dependent receiver-operator curves analysis. Forty-one of 205 patients (20%) had LGE; 12 of 205 (6%) died or had VT during follow-up; of these, 10 (83%) were in the LGE+ group. In the LGE+ group (1) the rate of death/VT per year was >20× higher than LGE- (4.9 versus 0.2%, P<0.01); (2) death/VT were associated with a greater burden of LGE (14±11 versus 5±5%, P<0.01) and right ventricular dysfunction (right ventricular EF 45±12 versus 53±28%, P=0.04). LGE burden was the best predictor of death/VT (area under the receiver-operating characteristics curve, 0.80); for every 1% increase of LGE burden, the hazard of death/VT increased by 8%.

Conclusions: Sarcoidosis patients with LGE are at significant risk for death/VT, even with preserved left ventricular ejection fraction. Increased LGE burden and right ventricular dysfunction can identify LGE+ patients at highest risk of death/VT.

Keywords: cardiac arrhythmias; cardiac magnetic resonance; cardiomyopathy; defibrillator; gadolinium; heart failure; sarcoidosis.

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Figures

Figure 1.
Figure 1.
Detection and quantification of late gadolinium enhancement. Images from patient with a history of transient binocular diplopia of unclear pathogenesis presenting with dyspnea on exertion and palpitations. Coronary angiography without obstructive coronary artery disease but with basal inferior wall motion abnormality. Cardiac magnetic resonance reveals mediastinal lymphadenopathy, normal left ventricular ejection fraction (63%), and late gadolinium enhancement as shown above. Lymph node biopsy with non-necrotizing granulomas. Left, A T1-weighted gradient-echo pulse sequence with a phase-sensitive inversion recovery reconstruction showing subepicardial distribution of late gadolinium enhancement in the septum (yellow arrows). Middle, Commercially available software was used to delineate late gadolinium enhancement. Right, Corresponding diastolic frame from steady-state free-precession cine imaging. Red areas denote regions in which the myocardial signal intensity is ≥5 SDs above the region designated as normal (white arrow) by the operator.
Figure 2.
Figure 2.
Kaplan–Meier curves demonstrating the impact of cardiac sarcoidosis on survival in the late gadolinium enhancement (LGE)+ (red) and LGE− (blue) groups. P value refers to logrank test LGE+ vs LGE− survival.
Figure 3.
Figure 3.
Univariate Cox proportional hazard models for the total population demonstrating that the presence of LGE has a hazard ratio of 24.5 (5.3–112.9; P<0.01) for death or ventricular tachycardia (VT). LVEDVi indicates left ventricular end-diastolic volume index; LVEF, left ventricular ejection fraction; LVESVi, left ventricular end-systolic volume index; RVEF, right ventricular ejection fraction; RVEDVi, right ventricular end-diastolic volume index; and RVESVi, right ventricular end-systolic volume index.
Figure 4.
Figure 4.
Univariate Cox proportional hazard models for the late gadolinium enhancement (LGE)+ group, demonstrating that hazard ratio of the burden of LGE for predicting death or ventricular tachycardia (VT) was 1.08 (1.02–1.14; P=0.01) equivalent to a 8% increase in the hazard of death or VT for each 1% increase in burden of LGE. LVEDVi indicates left ventricular end-diastolic volume index; LVEF, left ventricular ejection fraction; LVESVi, left ventricular end-systolic volume index; RVEF, right ventricular ejection fraction; RVEDVi, right ventricular end-diastolic volume index; and RVESVi, right ventricular end-systolic volume index.
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