Large dissecting sinus of Valsalva aneurysm creating a 'triple ventricle' appearance: case report.

BACKGROUND

Dissecting aneurysm of the interventricular septum (DAIS) is a rare congenital or acquired anomaly which can have a progressive course. We report a large DAIS incidentally detected by a routine echocardiogram.

CASE SUMMARY

An asymptomatic tricenarian was incidentally detected to a DAIS during a routine pre-operative evaluation. Multimodality imaging with cardiac CT, MRI, and aortic root angiograms confirmed the diagnosis. Work-up for infectious causes like syphilis and inflammatory causes like connective tissue disorders were negative. Patient is planned for surgical repair.

DISCUSSION

Dissecting aneurysm of the interventricular septum is a rare anomaly with a poor prognosis. The rupture of sinus of Valsalva aneurysm is the most common cause. Infections like syphilis and infective endocarditis, connective tissue disorders (Marfan syndrome and Ehler-Danlos syndrome), autoimmune diseases (Behcet's disease, ankylosing spondylitis, systemic lupus erythematosus, Takayasu's arteritis), bicuspid aortic valve with aortopathy, atherosclerosis, surgery, or trauma are the reported causes. Such dissecting aneurysms can have a progressive course, leading to right or left ventricular outflow tract obstructions, severe aortic regurgitation, and rupture into ventricular chambers, portending a grave prognosis. Hence, surgical correction is the norm.