Abstract PRKAG2 syndrome is a rare, early-onset, autosomal dominant, inherited lysosomal glycogen storage disease that develops with ventricular preexcitation syndrome, supraventricular arrhythmias, and cardiac hypertrophy. The disease is caused by mutations in the gene encoding the adenosine monophosphate-activated protein kinase (AMPK) protein, leading to glycogen accumulation in cardiomyocytes. Echocardiography is a noninvasive, widely available, and highly effective technique for identifying and quantifying left ventricular hypertrophy (LVH). Therefore, this review focuses mainly on echocardiographic patterns, describing the main alterations in patients […]