A 21-year-old female presents with progressive shortness of breath and typical exertional chest pain. Past medical history is significant for a known small PM-VSD diagnosed in childhood with no intervention required. Physical examination was notable for an ejection systolic murmur (grade IV/VI) at the left sternal border. Electrocardiogram revealed right ventricular hypertrophy (RVH) and right atrial enlargement.\nTransthoracic echocardiogram (TTE) while technically challenging, revealed a small (3 mm) PM-VSD with left to right shunting. In addition, RVH and a muscular septation within the RV cavity causing obstruction with a peak gradient of 70-80 mm Hg was noted. The pulmonic valve was normal. A transesophageal echocardiogram (TEE) confirmed the TTE findings (, ). Cardiac magnetic resonance (CMR) showed prominent RV outflow tract (RVOT) muscle bundles causing significant flow turbulence in systole (, ).\nThe patient underwent surgery using a transatrial approach with VSD closure and resection of the anomalous muscle bands. Recovery was uneventful with no significant residual gradient across the RV on follow-up echocardiography.
An asymptomatic 17-year-old male, with a history of surgical closure of PM-VSD at age 12, presents for routine follow-up. Physical examination was notable for an ejection systolic murmur (grade II/VI) at the left sternal border. ECG was normal. TTE revealed a RV muscular septation without significant obstruction (peak gradient of < 20 mm Hg). No residual VSD shunt was appreciated. CMR showed prominent RVOT muscle bundles causing no flow turbulence ( and , ). No action was taken due to the benign nature of DCRV in this patient.