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. 2022 Apr 26;22(1):192.
doi: 10.1186/s12872-022-02639-0.

Diagnosis, management, and outcome of cardiac sarcoidosis and giant cell myocarditis: a Swedish single center experience

Emanuele Bobbio  1   2 , Clara Hjalmarsson  1   2 , Marie Björkenstam  1   2 , Christian L Polte  3   2 , Anders Oldfors  4 , Ulf Lindström  5 , Pia Dahlberg  1   2 , Sven-Erik Bartfay  1   2 , Piotr Szamlewski  1   2 , Amar Taha  1   2 , Egidija Sakiniene  5 , Kristjan Karason  6 , Niklas Bergh  1   2 , Entela Bollano  7   8
Affiliations

Diagnosis, management, and outcome of cardiac sarcoidosis and giant cell myocarditis: a Swedish single center experience

Emanuele Bobbio et al. BMC Cardiovasc Disord. .

Abstract

Background: Cardiac sarcoidosis (CS) and giant cell myocarditis (GCM) are rare diseases that share some similarities, but also display different clinical and histopathological features. We aimed to compare the demographics, clinical presentation, and outcome of patients diagnosed with CS or GCM.

Method: We compared the clinical data and outcome of all adult patients with CS (n = 71) or GCM (n = 21) diagnosed at our center between 1991 and 2020.

Results: The median (interquartile range) follow-up time for patients with CS and GCM was 33.5 [6.5-60.9] and 2.98 [0.6-40.9] months, respectively. In the entire cohort, heart failure (HF) was the most common presenting manifestation (31%), followed by ventricular arrhythmias (25%). At presentation, a left ventricular ejection fraction of < 50% was found in 54% of the CS compared to 86% of the GCM patients (P = 0.014), while corresponding proportions for right ventricular dysfunction were 24% and 52% (P = 0.026), respectively. Advanced HF (NYHA ≥ IIIB) was less common in CS (31%) than in GCM (76%). CS patients displayed significantly lower circulating levels of natriuretic peptides (P < 0.001) and troponins (P = 0.014). Eighteen percent of patients with CS included in the survival analysis reached the composite endpoint of death or heart transplantation (HTx) compared to 68% of patients with GCM (P < 0.001).

Conclusion: GCM has a more fulminant clinical course than CS with severe biventricular failure, higher levels of circulating biomarkers and an increased need for HTx. The histopathologic diagnosis remained key determinant even after adjustment for markers of cardiac dysfunction.

Keywords: Cardiac sarcoidosis; Endomyocardial biopsy; Giant cell myocarditis; Heart failure; Inflammatory cardiomyopathy; Myocarditis.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
The number of new cases of CS and GCM diagnosed in the 5-year periods between 1991 and 2020
Fig. 2
Fig. 2
Kaplan–Meier curves for transplantation-free survival in patients with cardiac sarcoidosis and giant cell myocarditis
See this image and copyright information in PMC

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