Research

Retinal angiogenesis and PAH

Endothelial cell (EC) dysfunction is an important mediator of the vascular remodeling in PAH. While the etiology of PAH is unknown for most cases, there is strong evidence for a unique underlying systemic EC abnormality, leading to a specific vascular phenotype. There is growing evidence that patients with PAH have a systemic EC defect that physiologically and anatomically affects vascular beds outside the lungs. EC dysfunction is also a major mediator of diabetic retinopathy leading to retinal microvascular ischemia, hemorrhage and neovascularization. Changes in the retinal microcirculation often predates clinical relevant end-organ damage like myocardial infarction and stroke. New technologies like adaptive optics scanning light ophthalmoscopy (AOSLO) and confocal AOSLO (cAOSLO) have recently been used to identify earliest pre-clinical stages of diabetic retinopathy. In this project, we will use AOSLO and cAOSLO in PAH patients to investigate if we can detect microvascular changes in the retina that are due to a unique PAH-specific EC dysfunction and that differ from microvascular changes seen in diabetic or hypertensive retinopathy. We will further investigate the usefulness of AOSLO and cAOSLO as a new biomarker and risk stratification tool in PAH.