Inferior vena cava stenosis: Echocardiographic diagnosis in Marfan syndrome

Marfan syndrome is a genetic disease with variable clinical presentation. This case describes a 36-year-old lady who was diagnosed with Marfan syndrome based on revised Ghent criteria. She was found to have bicuspid aortic valve and sensorineural hearing loss. Inferior vena cava stenosis was suspected on echocardiography due to high velocity flow and visualization of a focal narrowing in the inferior vena cava proximal to hepatic vein entry. Inferior vena cava stenosis was confirmed by computed tomography. Echocardiographic features suggestive of inferior vena cava stenosis include detection of a focal narrowing and high turbulent flow, peak velocity > 1.5 m/s and S/D wave fusion on spectral Doppler.