Pulmonary arterial hypertension of vascular origin: Presentation of a rare case. [Hypertension artérielle pulmonaire d'origine vasculaire : présentation d'un cas rare]

We report a case of pulmonary hypertension linked to the scimitar syndrome. This is a rare pulmonary vascular malformation. This syndrome, well known of the pediatrics have also an adult form. Its clinical presentation is an insidious dyspnea associated with signs of right heart failure due to a moderate pulmonary hypertension or atrial septal defect. An awareness of this syndrome is important because its prognosis is good and its treatment is medical for most adult patients.