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A 4 year-old male presented with effort dyspnea, and was diagnosed as atrioventricular canal defects. This finding was confirmed by open heart surgery, and a congenital double orifice mitral valve was discovered. The septal defect was closed but the double orifice mitral valve was respected because of the absence of hemodynamic disturbance. We report this case with review of literature.

Congenital left ventricular aneurysm is rare, poorly understood and potentially lethal. It usually occurs in isolation. Its combination with mitral insufficiency is an uncommon entity. Because the literature on this kind of aneurysms consists of case reports and small series described especially in children, we report here an interesting and unique case of an adult aged 35 years bearing simultaneously a congenital left ventricular aneurysm and mitral insufficiency. Without medical past history, he consults for palpitations and dyspnoea. Once diagnosis was made by chest X-ray, echocardiography and computed tomography, an open heart surgery was necessary to prevent complications. Through a transmitral approach, aneurysmal exclusion was performed by closing the aneurysmal collar with a Dacron patch and mitral replacement was accomplished. At 12 months, follow-up was favourable without residual communication between aneurysm and ventricle or paraprosthetic leak., (© The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)