The cardiac anomalies include right, left or biventricular disease variants with a genetic background related to the phenotypic expression, where RV involvement is associated with the plakophilin-2 (PKP2) gene, LV implication with desmoplakin (DSP) and LMNA, while biventricular expression has a more diverse genetic background. The gene discussed is PKP2; the disease is glycogen storage disease VI.