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.
A 34-year-old male was evaluated for palpitation. Past medical history is significant for small PM-VSD years earlier with no intervention required. Physical examination was notable for an ejection systolic murmur (grade II/VI) over the left sternal border. ECG was unremarkable. TTE showed a small (4 mm) PM-VSD with left to right shunt and RV muscular septation causing no significant obstruction. TEE was confirmatory (, ). CMR showed prominent RVOT muscle bundles causing no significant flow turbulence (, ). No action was taken due to the benign nature of DCRV in this patient.