In a review by Porto et al. on the clinical spectrum of the PRKAG2 syndrome (PS), a rare, early onset autosomal dominant inherited disease characterized by ventricular pre-excitation, supraventricular arrhythmias and cardiac hypertrophy, the team aimed to describe the various features and clinical implications of PS after reviewing a total of 193 genetically confirmed patients and 13 different mutations of PRKAG2 gene. The gene discussed is PRKAG2; the disease is PRKAG2-related cardiomyopathy.