A 47-year-old female with pulmonary hypertension presented with headaches and vision changes progressing over 1 year. She had difficulty focusing because her vision was a “total blur” with black dots all over the visual fields. She had no double vision, sudden onset vision loss, pain with eye movement, or transient visual obscuration. Medications included sildenafil and visual changes worsened with increased sildenafil dose. On exam, her best-corrected visual acuity decreased to 20/30 in each eye. The pupil exam was normal without relative afferent pupillary defect. The ocular motility was normal and there was no misalignment.  Her fundus exam was normal with healthy optic nerves and maculae in each eye. Goldmann visual field showed full field in the right eye and generalized mild constriction in the left eye. MRI brain was normal. Full field electroretinography (ERG) showed normal amplitude, wave form, and timing. Multi-focal ERG showed blunting of the foveal peaks in both eyes, with focal depression in the macula, more prominent in the left eye. A diagnosis of presumed sildenafil associated maculopathy coincidental with primary headache syndrome was made. After discussion with her pulmonologist, sildenafil was discontinued. At 2 months follow-up, her headaches improved, and visual symptoms were stable. 

Vision changes can be separate from a headache syndrome. In our patient, sildenafil was associated with progressive vision loss due to maculopathy, which was unrelated to worsened primary headaches. In patient with pulmonary hypertension, the decision to discontinue sildenafil should be made with Pulmonology.