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.