Isolated Primary Cardiac Sarcoidosis Presenting as Acute Heart Failure

Abstract

A 65-year-old man was referred to our hospital due to an acute onset of dyspnea and persistent fever. Echocardiography revealed an ejection fraction (EF) of 25% with diffuse severe left ventricular (LV) dysfunction. 18F-fluorodeoxy glucose-positron emission tomography imaging showed significantly increased uptake by the LV and right ventricular walls, indicating active inflammation. The histologic findings of the endomyocardial biopsy specimens indicated the presence of epithelioid cell granuloma. The final diagnosis was thus cardiac sarcoidosis with acute inflammation. Five-months after the initiation of steroid therapy, echocardiography showed an EF of 50%. This is a rare case in which acute inflammation led to acute heart failure mimicking acute myocarditis.

Cardiac Sarcoidosis

“…16,18,19,[33][34][35][36] In our work, 71 of 110 patients (65%) presented with clinically isolated CS, which is close to the number (57%) reported by Okura et al 15 from an international registry. Although cardiac involvement is the only clinically apparent manifestation of sarcoidosis at presentation, hidden involvement of other organs can be found by 18 F-FDG PET or at autopsy.…”

“…16,18,19,[33][34][35][36] In our work, 71 of 110 patients (65%) presented with clinically isolated CS, which is close to the number (57%) reported by Okura et al 15 from an international registry. Although cardiac involvement is the only clinically apparent manifestation of sarcoidosis at presentation, hidden involvement of other organs can be found by 18 F-FDG PET or at autopsy. 3,4,6,17,27 In our isolated CS subgroup, silent extracardiac FDG uptake was common (Table 1), but still, 9 of the 30 patients undergoing whole-body PET (30%) had no signs of active sarcoidosis outside the heart.…”

“…We think that improved diagnostic methods, combined with better awareness of CS and heightened resolution in the pursuit of diagnosis, 17 may explain this change without a true increase in disease incidence. Of note, the largest step-up in the number of new diagnoses ( Figure 1) followed the introduction of Gd-MRI and 18 F-FDG PET into the routine clinical assessment of myocardial diseases in our country.…”

“…[7][8][9] CS is reported to involve only 2% to 5% of patients with systemic sarcoidosis, [7][8][9][10] but both autopsy studies [3][4][5] and modern cardiac imaging [11][12][13][14] suggest that clinically manifest cases may represent just the tip of the iceberg in CS. There is also much evidence indicating that sarcoidosis can be clinically confined to the heart, 3,6,[15][16][17][18][19] which may delay its detection. 17 …”

The Diagnostic Dilemma of Isolated Cardiac Sarcoidosis

“…Although histopathological confirmation is needed (3), the sensitivity of endomyocardial biopsies is less than 20% due to the random distribution of cardiac involvement. Fortunately, the case reported by Sugizaki et al (4) involved histological confirmation of CS on an endomyocardial biopsy, and that patient was successfully treated with corticosteroids. However, such cases are rare, and it is quite difficult to establish a clinical diagnosis of isolated CS.…”