Assessing the prevalence of extensive macular atrophy with pseudodrusen-like appearance in patients with rheumatic fever-associated valvular heart disease: a cross-sectional study

Abstract Background Extensive macular atrophy with pseudodrusen-like appearance (EMAP) is a rare retinal disorder characterized by bilateral macular atrophy and subretinal drusenoid deposits (SDDs), typically occurring earlier and progressing faster than age-related macular degeneration. A possible association between EMAP and rheumatic fever (RF) has been proposed, but its prevalence in this population remains unclear. Methods In this cross-sectional study, 118 patients (236 eyes) with valvular disease secondary to RF were prospectively screened at a tertiary cardiology clinic. EMAP was defined by vertically predominant macular atrophy with SDDs and exclusion of alternative diagnoses. Multimodal imaging included spectral-domain optical coherence tomography and fundus autofluorescence. Demographic, clinical, and cardiologic parameters were descriptively analyzed between patients with and without EMAP, and compared between those with and without SDDs using appropriate statistical tests. Results Two patients (1.69%; 95% confidence interval [CI], 0.47–5.97%) met diagnostic criteria for EMAP. Both were women aged 62 and 73 years, with RF onset during childhood and long disease duration (54 and 62 years). SDDs were identified in 12 patients (10.17%; 95% CI, 5.91–16.94%), including both EMAP cases. Patients with SDDs were significantly older (p = 0.007), had longer RF duration (p = 0.032), and received a greater cumulative benzathine penicillin prophylaxis burden (p = 0.029). Conclusions EMAP is uncommon among patients with RF-associated valvular disease, suggesting that additional genetic or environmental factors may be necessary for disease manifestation. The prevalence of SDDs highlights potential subclinical retinal involvement associated with chronic systemic inflammation in this patient population.