Echocardiography in inflammatory heart disease: A comparison of giant cell myocarditis, cardiac sarcoidosis, and acute non-fulminant myocarditis

Emanuele Bobbio; Johanna Amundsen; Anders Oldfors; Entela Bollano; Niklas Bergh; Marie Björkenstam; Marco Astengo; Kristjan Karason; Sinsia A Gao; Christian L Polte

doi:10.1016/j.ijcha.2023.101202

Echocardiography in inflammatory heart disease: A comparison of giant cell myocarditis, cardiac sarcoidosis, and acute non-fulminant myocarditis

Int J Cardiol Heart Vasc. 2023 Apr 7:46:101202. doi: 10.1016/j.ijcha.2023.101202. eCollection 2023 Jun.

Authors

Emanuele Bobbio^{1

2}, Johanna Amundsen³, Anders Oldfors^{4

5}, Entela Bollano^{1

2}, Niklas Bergh^{1

2}, Marie Björkenstam^{1

2}, Marco Astengo^{3

2}, Kristjan Karason^{1

6

2}, Sinsia A Gao^{3

2}, Christian L Polte^{3

7

2}

Affiliations

¹ Department of Cardiology, Sahlgrenska University Hospital, Gothenburg, Sweden.
² Institute of Medicine, The Sahlgrenska Academy at the University of Gothenburg, Gothenburg, Sweden.
³ Department of Clinical Physiology, Sahlgrenska University Hospital, Gothenburg, Sweden.
⁴ Department of Clinical Pathology, Sahlgrenska University Hospital, Gothenburg, Sweden.
⁵ Institute of Biomedicine, The Sahlgrenska Academy at the University of Gothenburg, Gothenburg, Sweden.
⁶ Department of Transplantation, Sahlgrenska University Hospital, Gothenburg, Sweden.
⁷ Department of Radiology, Sahlgrenska University Hospital, Gothenburg, Sweden.

Abstract

Background: Giant cell myocarditis (GCM) and cardiac sarcoidosis (CS) are, in contrast to acute non-fulminant myocarditis (ANFM), rare inflammatory diseases of the myocardium with poor prognosis. Although echocardiography is the first-line diagnostic tool in these patients, their echocardiographic appearance has so far not been systematically studied.

Methods: We assessed a total of 71 patients with endomyocardial biopsy-proven GCM (n = 21), and CS (n = 25), as well as magnetic resonance-verified ANFM (n = 25). All echocardiographic examinations, performed upon clinical presentation, were reanalysed according to current guidelines including a detailed assessment of right ventricular (RV) dysfunction.

Results: In comparison with ANFM, patients with either GCM or CS were older (mean age (±SD) 55 ± 12 or 53 ± 8 vs 25 ± 8 years), more often of female gender (52% or 24% vs 8%), had more severe clinical symptoms and higher natriuretic peptide levels. For both GCM and CS, echocardiography revealed more frequently signs of left ventricular (LV) dysfunction in form of a reduced ejection fraction (p < 0.001), decreased cardiac index (p < 0.001) and lower global longitudinal strain (p < 0.001) in contrast to ANFM. The most prominent increase in LV end-diastolic volume index was observed in CS. In addition, RV dysfunction was more frequently found in both GCM and CS than in ANFM (p = 0.042).

Conclusions: Both GCM and CS have an echocardiographic and clinical appearance that is distinct from ANFM. However, the method cannot further differentiate between the two rare entities. Consequently, echocardiography can strengthen the initial clinical suspicion of a more severe form of myocarditis, thus warranting a more rigorous clinical work-up.

Keywords: Cardiac sarcoidosis; Echocardiography; Giant cell myocarditis; Inflammatory heart disease; Myocarditis; Right ventricular dysfunction.