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Giant Muscular Ventricular Septal Defect (VSD) with Dual-Chamber Right Ventricle

Imaging findings

During a TAVR workup, CT incidentally revealed a large defect in the interventricular septum, accompanied by significant hypertrophy of the ventricular myocardium. The right ventricle appeared to have a 'dual-chamber' morphology, with the VSD affecting the moderator band.

Key takeaways

Giant muscular ventricular septal defects are rare congenital anomalies that can present in adulthood. In the setting of aortic stenosis and ventricular hypertrophy, these defects may lead to a 'dual-chamber right ventricle' effect, where the RV acts as a second chamber due to RV outflow obstruction, but may not show significant shunting or dilation of the RV itself. Multiple clefts in the septum are sometimes referred to as a sawtooth septum.

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