Transcatheter closure of ruptured sinus of Valsalva aneurysm causing Fontan circulation failure

Congenital sinus of Valsalva aneurysm is a rare cardiac malformation that usually becomes symptomatic as a consequence of intracardiac or extracardiac rupture. It is difficult to suspect in association with complex cardiac defects and its rupture may be misdiagnosed as progressive aortic regurgitation. This case report refers to a patient with tricuspid atresia submitted to Fontan procedure five years previously, in whom a sinus of Valsalva aneurysm rupture into the accessory ventricular chamber caused rapidly progressive heart failure. The malformation was suspected by echocardiography and treated by percutaneous implantation of an Amplatzer duct occluder, with immediate improvement of the patient's clinical and functional status.