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.
A 58-year-old female was evaluated for shortness of breath on moderate exertion. Cardiac history is that of a known small asymptomatic PM-VSD diagnosed two decades earlier. Physical examination was notable for a harsh ejection systolic murmur (grade III/VI) at the left sternal border. Electrocardiogram (ECG) revealed RVH. TTE revealed a large (14 mm) PM-VSD with left to right shunt. RVH and muscular septation of the RV causing mid-ventricular obstruction with a peak gradient of -75-80 mm Hg was noted. CMR showed prominent RVOT muscle bundles causing flow turbulence with significant obliteration in systole (area -0.6 cm2) ( and , ). Cardiac catheterization confirmed the presence of DCRV, with an 85 mm Hg pressure gradient between the proximal and distal chambers. A decision was made to proceed with surgical repair; however, the patient declined further intervention.