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

BMC Cardiovascular Disorders

Entela Bollano¹  Marie Björkenstam¹  Emanuele Bobbio¹  Clara Hjalmarsson¹  Pia Dahlberg¹  Sven-Erik Bartfay¹  Amar Taha¹  Piotr Szamlewski¹  Niklas Bergh¹  Anders Oldfors²  Christian L. Polte³  Ulf Lindström⁴  Egidija Sakiniene⁴  Kristjan Karason⁵ 

[1] Departments of Cardiology, Sahlgrenska University Hospital;Departments of Clinical Pathology, Sahlgrenska University Hospital;Departments of Clinical Physiology and Radiology, Sahlgrenska University Hospital;Departments of Rheumatology, Sahlgrenska University Hospital;Transplant Institute, Sahlgrenska University Hospital;

关键词: Cardiac sarcoidosis;    Giant cell myocarditis;    Inflammatory cardiomyopathy;    Myocarditis;    Endomyocardial biopsy;    Heart failure;   

DOI  :  10.1186/s12872-022-02639-0

来源: DOAJ

【 摘 要 】

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.

【 授权许可】

Unknown