Migraine with aura following atrial septostomy for pulmonary arterial hypertension.

BACKGROUND: A 34-year-old woman with known familial pulmonary arterial hypertension presented with syncope. Despite medical therapy with an endothelin-receptor antagonist and a phosphodiesterase inhibitor, the patient had NYHA class III symptoms, with exertional dyspnea. Right heart catheterization revealed severe pulmonary hypertension (right atrial pressure 15 mmHg, right ventricular pressure 80/15 mmHg, pulmonary artery pressure 80/35 mmHg, mean pulmonary artery pressure 52 mmHg). The patient underwent balloon atrial septostomy, creating a right-to-left shunt. Although she had no history of headaches or migraine attacks, the patient developed a migraine headache with aura on the third day after the procedure. Migraine attacks recurred for the next 2 days, but symptoms were relieved with simple analgesia. INVESTIGATIONS: Physical examination, electrocardiography, laboratory testing, 6-min-walk test, right heart catheterization, chest radiography, transesophageal echocardiography, transthoracic echocardiography. DIAGNOSIS: Migraine with aura following atrial septostomy. MANAGEMENT: Bosentan, sildenafil, furosemide, spironolactone and warfarin therapy, atrial septostomy, and paracetamol therapy for migraine.