The Retinal Vessel Density as a New Vascular Biomarker in Multisystem Involvement in Fabry Disease: An Optical Coherence Tomography Angiography Study

• Main Authors: Gilda Cennamo, Daniela Montorio, Ciro Santoro, Sirio Cocozza, Letizia Spinelli, Teodolinda Di Risi, Eleonora Riccio, Camilla Russo, Giuseppe Pontillo, Roberta Esposito, Massimo Imbriaco, Antonio Pisani
• Format: Article
• Language: English
• Published: MDPI AG 2020-12-01
• Series: Journal of Clinical Medicine
• Subjects: Fabry disease; optical coherence tomography angiography; retinal vessel density; echocardiography; peak arterial pulmonary pressure; left atrial volume index
• Online Access: https://www.mdpi.com/2077-0383/9/12/4087

In this study, we evaluated the possible relationship between the changes in retinal vessel density (VD) by optical coherence tomography angiography (OCTA) and the vascular alterations involving renal, cardiovascular and central nervous systems in patients affected by Fabry disease (FD). In 50 FD patients, the retinal superficial capillary plexus (SCP) and deep capillary plexus (DCP) in macular region were evaluated by OCTA examination. The patients also underwent a brain magnetic resonance imaging scan, renal and echocardiographic examinations with quantification of systolic pulmonary arterial pressure (PAPs) and left atrial volume index (LAVi). The VD of SCP and DCP was inversely related with E/e’ ratio, LAVi, interventricular septal thickness, global longitudinal strain (GLS) and PAPs (<i>p</i> < 0.05). No relationship was found, with a multivariate analysis, between OCTA parameters and kidney function and neuroradiological signs of central nervous system involvement. OCTA could be a new vascular biomarker in FD, revealing a strong correlation between retinal capillary damage and myocardial impairment, possibly preceding both renal dysfunction and cerebrovascular involvement.