Right Ventricle–Dominant Cardiac Sarcoidosis Diagnosed Using a Multimodal Approach

Figures

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  Visual Summary. Right Ventricular–Dominant Cardiac Sarcoidosis

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  Figure 1. Electrocardiographic and Echocardiographic Images of the Patient (A) Electrocardiogram showing first-degree atrioventricular block, complete right bundle block, and left anterior fascicular block, along with premature ventricular contractions of left bundle branch block morphology, negative T waves in V1 to V5 leads, and epsilon waves in V1 to V3 leads. The image on the right is an enlarged view of the V1 lead, where epsilon waves are more clearly visible, emphasizing their significance. (B and C) Transthoracic echocardiography showing a parasternal long-axis view (B) and an RV-focused apical 4-chamber view (C) with RV enlargement and focal thickening of the RV wall. Ao = aorta; LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle.

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  Figure 2. Cardiac imaging of the Patient, Including Computed Tomography, Magnetic Resonance Imaging, and PET-CT (A and B) Cardiac computed tomography showing mediastinal and hilar lymphadenopathy. Pink arrow indicates swollen lymph node. (C) Cardiac computed tomography demonstrating the RV enlargement and focal thickening of the RV wall. (D) Short-axis view of the cardiac computed tomography showing dilatation and focal thickening of the RV. (E) Short-axis late iodine enhancement (LIE) imaging of the same cross-section as in (D), showing extensive LIE on the thickened RV free wall and epicardial side of the ventricular septum (pink arrows). (F) Cardiac magnetic resonance image showing extensive late gadolinium enhancement on the thickened RV free wall and the epicardial side of the ventricular septum, corresponding to the same region as the LIE observed on cardiac computed tomography (pink arrows). (G) FDG-PET image in the short-axis view corresponding to the same cross-section as (E and F), showing abnormal 18F-fluorodeoxyglucose uptake in the thickened RV free wall and the epicardial side of the interventricular septum (pink arrows). LV = left ventricle; RV = right ventricle; PET-CT =…

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  Figure 3. 18F-Fluorodeoxyglucose Positron Emission Tomography Scan of the Patient (A) 18F-fluorodeoxyglucose positron emission tomography at the time of diagnosis revealing abnormal 18F-fluorodeoxyglucose uptake in the heart, predominantly in the thickened right ventricular wall and the basal region of the ventricular septum, as well as in the mediastinal, hilar, and right supraclavicular fossa lymph nodes. (B) 18F-fluorodeoxyglucose positron emission tomography scan performed 4 months after the initiation of steroid therapy showing the resolution of hyperaccumulation in both the cardiac region and lymph nodes.

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  Figure 4. Subendocardial Myocardial Biopsy Performed From the Right Ventricular Septum (A) Low-magnification view of the myocardial tissue stained with hematoxylin and eosin (HE), showing the overall architecture of the myocardium. (B) High-magnification view of the myocardial tissue stained with HE, demonstrating the formation of noncaseating epithelioid cell granulomas (yellow arrow) surrounded by lymphocytic infiltration.

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  Figure 5. Holter Monitoring of the Patient Monitor electrocardiogram showing frequent multiform premature ventricular contractions and nonsustained ventricular tachycardia.