Cardiac Sarcoidosis Mimicking Septal Tumor with Intermittent Complete Atrioventricular Block

Abstract

A 52-year-old woman with intermittent complete atrioventricular (AV) block detected on exercise was admitted to the hospital. Echocardiography revealed lesions on the right ventricular side of the interventricular septum and free wall of the basal inferolateral area. Gadolinium-enhanced cardiovascular magnetic resonance (CMR) imaging revealed the mass and wall thickening at the same locations with late gadolinium enhancement (LGE). Focal uptake at the septal lesion was detected using 67Ga scintigraphy. Focal on diffuse intense uptake in the lesions was observed on Fluorine-18 fluorodeoxyglucose (18F-FDG) positron emission tomography (PET) imaging. Whole-body CT and laboratory evaluations uncovered no signs of malignant tumors in other organs. Ophthalmologic evaluation revealed ophthalmologic sarcoidosis. Although the result of endomyocardial biopsy was negative, the presence of cardiac sarcoidosis was strongly suggested on the basis of the new Japanese guidelines published in 2017. AV conduction disturbance and tracer accumulation on 67Ga scintigraphy completely disappeared after 2 weeks of steroid therapy. The size of mass, inferolateral wall thickness in echocardiography and CMR, and standardized uptake value (SUV) of the masses on 18F-FDG PET also decreased over time.

Recovery of Transient High-Grade Atrioventricular Block Managed With Corticosteroid Therapy Without Device Implantation in Newly Diagnosed Cardiac Sarcoidosis: A Case Report

“…As previously noted, while some authors have argued that the possibility of disease progression should warrant ICD implantation even if the high-grade or complete AV conduction block transiently recovers, this stance appears to be challenged by emerging case report literature [12][13][14][15][16]. Our patient's successful response to steroid therapy, though, should not be entirely surprising given that his patient profile was consistent with known factors associated with potential improvement in response to therapy in CS patients presenting with AVBs [11,12].…”

“…As mentioned before, the patient has not received an EPS and remains asymptomatic as well. Further, most cases of AVB recovery on steroid therapy have occurred in patients with normal LV dysfunction like our patient [9,14,15]. Thus, while the initial management strategy included both steroid therapy and ICD implantation, recovery of his transient AVB, as well as the patient's reluctance toward an invasive procedure, led to a shared decision to continue with only steroid therapy so long as the patient had a reliable follow-up with the proper specialists.…”

“…With regard to transient AVB, authors of a 2018 case described a patient with newly diagnosed CS complicated by intermittent complete AVB that recovered after two weeks of prednisolone 30 mg daily who also did not receive device implantation [14]. Finally, there have been a few cases reported whereby clinicians have administered high-dose steroid therapy, also known as pulse steroid therapy, for cases of complete AVB that lead to recovery as well and again without pursuing device therapy [15,16].…”

Primary Cardiac Angiosarcoma

“…Furthermore, cardiac tumors also need to be differentiated from other rare diseases to improve the accuracy of clinical diagnosis. 9) Angiosarcoma is the most common histologic subtype of primary cardiac malignant tumors among adults, 1,2) and it usually occurs in men aged 65 years, with a male-to-female ratio of around 2-3:1. 4,7) The most vulnerable site is in the right atrium, with 74% of primary cardiac angiosarcomas occurring in the right atrium and often involving adjacent structures.…”

Multiple Intramyocardial Masses in an Otherwise Healthy 35-Year-Old Woman

“… 1 Sarcoidosis presenting as a single intramyocardial mass mimicking a cardiac tumour is uncommon. 2 On review of previously reported cases, this case appears to be an extremely rare instance of multiple masses mimicking tumours. This case highlights the utility of multimodality imaging and biopsy in the work-up of this unusual presentation.…”