Active right ventricular cardiac sarcoidosis

Case contributed by Dr Azza Elgendy

Presentation

Sudden onset of ventricular arrhythmia.

Patient Data

Age: 25 years
Gender: Male

Axial SSFP shows irregular hypertrophy of the free wall of the right ventricle in addition to marked hypertrophy of the interventricular septum, predominantly at the inferior septum. A post contrast Dixon shows marked mediastinal, hilar and paratracheal adenopathy. Dynamic perfusion shows no early enhancement of the areas of hypertrophy. Delayed PSIR (phase sensitive inversion recovery) shows marked delayed enhancement of the areas of hypertrophy predominantly at the inferior septum. 

CT scan shows a marked mediastinal, hilar and paratracheal lymphadenopathy in addition to scattered micronodular opacities in the lungs, most notably in the superior segment of the right lower lobe. 

There is marked septal hypertrophy favoring the right ventricular septum. Intense delayed enhancement corresponding to the areas of hypertrophy. Additionally, there is transmural enhancement at the left ventricular apex.

The areas of hypertrophy do not demonstrate arterial phase enhancement on perfusion imaging.

Histopathology

Subcarinal lymph node fine-needle aspiration shows non-caseating granulomatous inflammation. Special stains negative for fungal organisms and acid-fast bacilli.  

Annotated image

The left hand image is a 4 chamber post contrast phase sensitive inversion recovery (PSIR), showing marked enhancement of the right ventricular free wall and insertional septum. There is also involvement of the true apex.

The right hand photo is a set of 2 images: A. is short axis SSFP shows hypertrophy of the septum and the right ventricle free wall. B. Delayed short axis PSIR shows marked enhancement of the areas of hypertrophy.

Case Discussion

This case demonstrates cardiac sarcoidosis manifesting predominantly in the right ventricle in addition to lung involvement.

MRI is the radiological modality of choice to evaluate for cardiac sarcoidosis. Typical findings include nodular mid-wall hyperintense foci on black blood T2-weighted imaging, areas of focal myocardial thickening and delayed enhancement, which may be seen in various patterns, but is most commonly either mid-wall or transmural.

Dynamic perfusion can be a useful adjunct to differentiate sarcoidosis from infiltrative masses such as lymphoma. 

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Case information

rID: 55596
Case created: 16th Sep 2017
Last edited: 9th Oct 2017
System: Cardiac
Inclusion in quiz mode: Included

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