Cardiopulmonary complications are infrequent in patients with ventriculoatrial shunts (VAS). We report the case of an 18-year-old man with congenital hydrocephalus who developed pulmonary arterial hypertension (PAH) in the long-term follow-up after a VAS procedure. He presented with progressive exertional dyspnea, stabbing chest pain and acral cyanosis. Echocardiography showed severe PAH and a digitiform mass adhered to the distal catheter. A ventilation/perfusion scan suggested chronic pulmonary embolisms. Anticoagulation with intravenous heparin was started and thrombus resolution was achieved, but PAH remained. It is necessary to bear in mind complications linked to VAS when treating a patient with this device.
