This PRKAG2 cardiomyopathy cohort demonstrated a clinical profile dominated by cardiac manifestations, including biventricular hypertrophy (19/22 patients with left and right ventricular hypertrophy), without evidence of skeletal myopathy as seen in patients with the PRKAG2 syndrome with the Asn488Ile mutation27. The gene discussed is PRKAG2; the disease is PRKAG2-related cardiomyopathy.