Purpose: The aim of this study was to report the case of paracentral acute middle maculopathy (PAMM) associated with pulmonary arterial hypertension (PAH), and to highlight its potential as an early indicator of systemic hypoxemia. Methods: This is a case report. Results: A 76-year-old Japanese woman presented with bilateral visual disturbances and was found to have PAMM lesions on optical coherence tomography. Her initial best-corrected visual acuity (BCVA) was 20/25 in both eyes. Over the three-year follow-up, recurrent PAMM lesions appeared in various retinal locations, coinciding with progressive visual decline and worsening cardiopulmonary function. The patient experienced repeated hospitalizations due to systemic deterioration. After the initiation of home oxygen therapy, no new PAMM lesions were observed during follow-up; however, progressive vascular dropout in the central macula led to irreversible vision loss. Her BCVA declined to 20/200 in the right eye and 20/30 in the left eye at the last visit. Conclusion: The definitive diagnosis of PAH requires invasive testing and is therefore frequently delayed. This case suggests that PAMM may serve as a useful ocular indicator prompting early systemic evaluation. Recognizing PAMM lesions could facilitate earlier diagnosis and intervention in patients with underlying hypoxemic conditions such as PAH.
Tanikawa et al. (Wed,) studied this question.