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Prognostic value of cardiovascular magnetic resonance in patients with biopsy-proven systemic sarcoidosis

  • Cardiac
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Abstract

Objectives

As prognosis in sarcoidosis is determined by cardiac involvement, the objective was to study the added value of cardiovascular magnetic resonance (CMR) in risk stratification.

Methods

In 114 patients (48 ± 12 years/52% male) with biopsy-proven sarcoidosis, we studied the value of clinical and CMR-derived parameters to predict future events, using sustained ventricular tachycardia, ventricular fibrillation, aborted cardiac death, implantable cardioverter-defibrillator (ICD) placement with appropriate shocks, hospitalization for heart failure, and death as composite endpoint. Median follow-up after CMR was 3.1 years (1.1–5.7 years).

Results

The ejection fraction (EF) was 58.2 ± 9.1% and 54.7 ± 10.8% for left ventricle (LV) and right ventricle (RV), respectively. LV late gadolinium enhancement (LGE) was present in 40 patients (35%) involving 5.1% of the LV mass (IQR, 3.0–12.0%), with concomitant RV involvement in 12 patients (11%). T2-weighting imaging and/or T2 mapping showed active disease in 14 patients. The composite endpoint was reached in 34 patients, with 7 deaths in the LGE-positive group (17.5%), versus two deaths in the LGE-negative group (2.7%) (p = 0.015). At univariate analysis, RVEF (p = 0.009), pulmonary arterial pressure (p = 0.002), and presence of LGE (p < 0.001) and LGE (% of LV) (p < 0.001) were significant. At multivariate analysis, only presence of LGE and LGE (% of LV) was significant (both p = 0.03). At Kaplan-Meier, presence of LGE and an LGE of 3% predicted event-free survival and patient survival. We found no difference in active versus inactive disease with regard to patient survival.

Conclusion

Myocardial enhancement at LGE-CMR adds independent prognostic value in risk stratification sarcoidosis patients. In contrast, clinical as well as functional cardiac parameters lack discriminative power.

Key Points

• Sarcoidosis often affects the heart.

• Comprehensive CMR, including T2 imaging and LGE enhancement CMR, allows to depict both active and inactive myocardial damage.

• Patient prognosis in sarcoidosis is determined by the presence and severity of myocardial involvement at LGE CMR.

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Abbreviations

CMR:

Cardiovascular magnetic resonance

EF:

Ejection fraction

ICD:

Implantable cardioverter-defibrillator

ISP:

IntelliSpace Portal

LGE:

Late gadolinium enhancement

LV:

Left ventricle

LVEDVi:

Normalized LV end-diastolic volume

LVESVi:

Normalized LV end-systolic volume

LVEF:

Left ventricular ejection fraction

LVMi:

Normalized LV mass

ROC:

Receiver operating characteristic

RV:

Right ventricle

RVEDVi:

Normalized RV end-diastolic volume

RVEF:

Right ventricular ejection fraction

SCD:

Sudden cardiac death

STIR:

Short tau inversion recovery

TSE:

Turbo spin echo

VF:

Ventricular fibrillation

VT:

Ventricular tachycardia

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Funding

The authors state that this work has not received any funding.

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Author notes

  1. Louis Flamée and Rolf Symons contributed equally to this work.

Authors and Affiliations

  1. Department of Radiology, University Hospitals Leuven, Leuven, Belgium

    Louis Flamée, Rolf Symons, Tom Dresselaers & Jan Bogaert

  2. Department of Nuclear Medicine and Molecular Imaging, University Hospitals Leuven, Leuven, Belgium

    Ganna Degtiarova

  3. Department of Nuclear Medicine, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain (UCL), Brussels, Belgium

    Olivier Gheysens

  4. Department of Respiratory Medicine, University Hospitals Leuven, Leuven, Belgium

    Wim Wuyts

  5. Department of Cardiovascular Sciences, KU Leuven, University of Leuven, Herestraat 49, Leuven, Belgium

    Johan Van Cleemput

  6. Department of Imaging and Pathology, KU Leuven, University of Leuven, Herestraat 49, 3000, Leuven, Belgium

    Jan Bogaert

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  1. Louis Flamée
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Corresponding author

Correspondence to Jan Bogaert.

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Guarantor

The scientific guarantor of this publication is Jan Bogaert.

Conflict of interest

The authors of this manuscript declare no relationships with any companies whose products or services may be related to the subject matter of the article.

Statistics and biometry

One of the authors has significant statistical expertise.

Informed consent

Written informed consent was waived by the Institutional Review Board.

Ethical approval

Institutional Review Board approval was obtained.

Study subjects or cohorts overlap

One case report has been published on an asymptomatic patient showing progressive disease at repeated CMR follow-up. This case report has no influence of the results of this study. Degtiarova G et al EHJ case reports Doi: https://doi.org/10.1093/ehjcr/ytz099

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• Performed at one institution

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Flamée, L., Symons, R., Degtiarova, G. et al. Prognostic value of cardiovascular magnetic resonance in patients with biopsy-proven systemic sarcoidosis. Eur Radiol 30, 3702–3710 (2020). https://doi.org/10.1007/s00330-020-06765-1

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  • DOI: https://doi.org/10.1007/s00330-020-06765-1

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