Carney complex featuring giant intracardiac biatrial myxoma in an adolescent girl

A 14-year-old girl presented with rapidly progressive pulmonary insufficiency requiring invasive mechanical ventilation. Medical history included extreme fatigue, undulating fever, and pigmented lesions of the skin on both legs. Echocardiography revealed giant intracardiac masses in both atriae protruding into both ventricles (Panel A). Left ventricular inflow was almost succumbed. The diagnosis was confirmed by computed tomography (Panel B), simultaneously excluding other causes for cardio-respiratory failure. The patient underwent emergency surgery through sternotomy with cardiopulmonary bypass (Panel C). Complete removal of the tumour, originating from the interatrial septum, was performed, and the septal defect closed with a pericardial patch (Panels D, E). Mitral valve ring annuloplasty was performed due to unmasked secondary severe mitral insufficiency. The patient recovered well and was discharged 3 weeks after the procedure. Histopathological examination confirmed the presence of a myxoma. During the post-operative period, the girl’s mother was...