<h4>Background</h4>Myosin inhibitors represent a novel therapeutic strategy for obstructive hypertrophic cardiomyopathy (oHCM), directly targeting sarcomeric hypercontractility to reduce left ventricular outflow tract obstruction.<h4>Case summary</h4>Three patients with symptomatic oHCM enrolled in Italy's Early Access Program for mavacamten underwent 2D and 3D echocardiographic assessment at baseline and at 6 months. Here, MYH14 is linked to hypertrophic cardiomyopathy.