Presentation
Palpitations, one episode of syncope, abnormal ECG changes.
Patient Data
Functional analysis
RV-EDVI: ~135mL/m2 [47-107 mL/m2]
RV-EF: ~35%
LV-EF: ~51%
Findings
The right ventricle is dilated. There is thinning of the right ventricular wall with a corrugated appearance. The right ventricular myocardium is replaced by fatty tissue. There is dilatation of the right ventricular outflow tract. There is hypokinesia of the right ventricle and bulging or mild regional dyskinesia of the free right ventricular wall.
Fatty tissue is also seen in left ventricular myocardium involving basal anterolateral, basal inferolateral, mid anterolateral and inferolateral segments.
A delayed contrast-enhanced scan shows late gadolinium enhancement of the right ventricular wall. There is also enhancement of the left ventricular wall's basal anterolateral, basal inferolateral, mid anterolateral, and inferolateral segments.
Case Discussion
Patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) commonly present with palpitations, syncope, or sudden cardiac arrest due to their propensity for ventricular ectopy arising from the structurally abnormal right ventricle.
The diagnosis of ARVC is based on the presence of major and minor criteria. These criteria encompass structural, histologic, ECG, arrhythmic, and genetic factors, however, these criteria lacked sensitivity 1.
The new Padua criteria emphasize late gadolinium enhancement at cardiac MRI as a key feature in diagnosis and imaging-based tissue characterization 2. This case is a fibrofatty variant characterized by significant thinning of the right ventricular wall, hypokinesia and the involvement of the left ventricular myocardial wall.
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