Korean J Radiol.  2015 Jun;16(3):668-672. 10.3348/kjr.2015.16.3.668.

Right Ventricular Cardiomyopathy Meeting the Arrhythmogenic Right Ventricular Dysplasia Revised Criteria? Don't Forget Sarcoidosis!

• Vasaturo S
• Ploeg DE
• Buitrago G
• Zeppenfeld K
• Veselic-Charvat M
• Kroft LJ

• KMID: 2155539
• DOI: http://doi.org/10.3348/kjr.2015.16.3.668

Abstract

A 53-year-old woman was referred for ventricular fibrillation with resuscitation. A CT-angiography showed signs of a right ventricular enlargement without obvious cause. A cardiac MRI demonstrated a dilated and hypokinetic right ventricle with extensive late gadolinium enhancement. Arrhythmogenic right ventricular dysplasia (ARVD) was suspected according to the "revised ARVD task force criteria". An endomyocardial biopsy was inconclusive. The patient developed purulent pericarditis after epicardial ablation therapy and died of toxic shock syndrome. The post-mortem pathologic examination demonstrated sarcoidosis involving the heart, lungs, and thyroid gland.

Keyword

Sarcoidosis; ARVD; Cardiomyopathy; MRI; Late enhancement; Right ventricle; Pathology

MeSH Terms

Arrhythmogenic Right Ventricular Dysplasia/*diagnosis
Female
Heart Ventricles/pathology
Humans
Lung/pathology
Magnetic Resonance Imaging
Middle Aged
Myocardium/pathology
Sarcoidosis/*diagnosis
Thyroid Gland/pathology
Ventricular Fibrillation/diagnosis/*etiology

Figure

• 

  Fig. 1 Cardiac sarcoidosis mimicking arrhythmogenic right ventricular dysplasia in 53-year-old woman. A. Pulmonary CT angiography on admission. Slightly enlarged mediastinal lymph nodes, aspecific finding (arrows, left panel). Bilateral dependent atelectasis but no other pulmonary abnormalities and no pulmonary embolism (left and middle panel). Enlarged right heart, with bowing of interventricular septum towards LV (right panel). B. MRI in axial orientation. Upper level (left panel), middle level (middle panel), and lower level (right panel) MRI showing bright LGE (arrows) in large parts of RV and patchy subepicardial LGE in interventricular septum and LV. LGE = late gadolinium enhancement, LV = left ventricle, RV = right ventricle C. Short-axis MRI images (upper row) compared with gross anatomic sections (lower row). Note perfect correlation between high signal LGE spots (arrows) on MRI at RV wall and apical septum and sarcoidosis granuloma (arrows) on pathology. Base (left panels), mid-ventricular (middle panels), and apex (right panels). D. Histology showing infiltration of histiocytes between myocardial fibers with formation of confluent granulomas and multinucleated giant cells, diagnostic for sarcoidosis (hematoxylin and eosin stain, × 100). Fibrosis and sparse lymphocytic infiltration are present. LGE = late gadolinium enhancement, LV = left ventricle, RV = right ventricle

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