Giant cell myocarditis causing sudden death in a patient with sarcoidosis

Ziegler, John P.; Batalis, Nicholas I.; Fulcher, James W.; Ward, Michael E.

doi:10.4322/acr.2020.238

Cited by 4 publications

(1 citation statement)

References 21 publications

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“…How the clinical differences relate to variable host immunological factors or to different environmental exposures is still unclear [ 22 ]. There have been several reports of patients possibly having both conditions [ 25 , 26 ]. Biopsy evidence of extracardiac sarcoidosis was reported in 5–10% of patients with a cardiac biopsy suggesting GCM [ 8 , 27 ].…”

Section: Discussionmentioning

confidence: 99%

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

Bobbio

Hjalmarsson

Björkenstam

et al. 2022

BMC Cardiovasc Disord

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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.

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Section: Discussionmentioning

confidence: 99%