RESUMO
A sinus of Valsalva aneurysm (SOVA) is a rare cardiac defect in which the aortic root area between the aortic annulus and the sinotubular junction is dilated. We present a case of acute liver failure (ALF) in a 21-year-old man secondary to ruptured SOVA inducing severe ischemic hepatitis. The patient presented clinically with classical ALF. The liver ultrasound reported hepatomegaly with pulsatile portal flow and dilated hepatic veins. A transthoracic echocardiogram revealed focal aneurysmal dilatation of the aortic root with flow across the aneurysm toward the right atrium and elevated right chambers pressures. The surgical repair of the non-coronary SOVA was successful, and post-operatively, liver transaminases improved, and ALF resolved. Given that ruptured SOVA can be surgically repaired, hepatologists should be aware of this diagnosis in a young patient with ALF.