Case presentation
A 14-year-old Japanese girl with postural orthostatic tachycardia syndrome (POTS) was referred to our hospital because of persistent headache for five months. She had no medical history other than POTS, and her developmental milestones were normal. Her brother had a history of POTS. The headache was located at the bilateral temporal regions, was aggravated by physical activity, followed by photophobia, and was not accompanied by disturbances in vision, sensation, or speech. The pain and fatigue worsened in the morning. The frequency of pain was at least 15 to 20 days a month, and the duration of pain was 8 to 12 hours. The laboratory data showed no abnormalities, and brain magnetic resonance imaging showed no abnormal findings. The active standing test revealed that the heart rate increase was still >30 beats per minute within 10 minutes without decreasing blood pressure. We diagnosed her headaches as being of the migraine type and also associated with POTS. We initiated treatment with midodrine hydrochloride and propranolol hydrochloride. After six months of treatment, the frequency of headaches was reduced to a few times a month, and the duration improved to less than three hours.
Until eight months later, the frequency of headaches remained almost the same, and her school life was not affected by the headaches, but then the headaches gradually worsened, and she began to have more than 15 episodes of headache per month. Furthermore, she could hardly go to school. In order to reduce the burden, it was decided to transfer her to a school closer to home. Even during that period, she was not using analgesics more than nine times a month.
At 18 years old, four years after the onset of chronic headaches, ASD and right ventricular enlargement were diagnosed after an echocardiogram was performed to investigate transient tachycardia. The size of the ASD was 7.5 mm, and the Qp/Qs ratio was 1.32. She underwent transcatheter closure for ASD with right ventricular enlargement using the Amplatzer® device. On the fifth day after the transcatheter ASD closure, she remarked that she had no headache at all. After returning to school one week after transcatheter ASD closure, the headaches were limited to a few times a month, and they spontaneously resolved within a duration of 30 minutes. However, her heart rate increase was still >30 beats per minute within 10 minutes of the active standing test after the surgery. At the outpatient visit six months later, she still had headaches for about an hour at a time, once or twice a month. She was not late or absent from school.