title: Ophthalmological consequences of cyanotic congenital heart disease: vascular parameters and nerve fibre layer.
abstract: BACKGROUND
  This study investigated the long-term ophthalmological consequences of cyanotic congenital heart disease (CHD).
  
  
  METHODS
  Cross-sectional study, tertiary referral setting.
  
  
  METHODS
  Thirteen adults with cyanotic CHD (40 ± 4 years). Age- and sex-matched healthy controls underwent aspects of the protocol.
  
  
  METHODS
  Cyanosed subjects had a full ophthalmic examination, visual fields, scanning laser ophthalmoscopy and optical coherence tomography to assess retinal nerve fibre layer (RNFL), retinal photography and cerebral magnetic resonance imaging (MRI).
  
  
  METHODS
  RNFL thickness and quantitative analysis of retinal vessels with fractal dimension, branching and central retinal arterial equivalent (CRAE) and central retinal venous equivalent (CRVE).
  
  
  RESULTS
  No abnormalities of anatomy, motility, intraocular pressure or anterior segments were detected apart from one subject who had bilateral cataracts. Corrected visual acuity was normal in all but one cyanosed subject. Clinical examination revealed dilated retinal vasculature in 12/13 cyanosed subjects and increased tortuosity in 8/13. In the setting of cyanosis, skeletonized retinal arterial and venous beds had higher fractal dimension and increased branching (P ≤ 0.01, n = 11 for all); retinal vessels were dilated (CRAE: 227 vs. 183, n = 11, P < 0.0001; CRVE: 254 vs. 221, n = 11, P = 0.01). Visual fields showed scotomas in two subjects associated with RNFL thinning. No disc oedema was detected. 6/13 subjects' RNFL thickness fell below the normal 95% confidence interval in at least one sector without explanatory cerebral pathology (P < 0.0001, n = 13). Mean RNFL thickness correlated with MRI cerebral white matter volume (R = 0.67, P = 0.035).
  
  
  CONCLUSIONS
  Cyanosed subjects had vessel dilatation, increased branching and tortuosity. RNFL and visual field thresholds were reduced suggesting impaired neuro-ophthalmological functioning.
authors: Department of Cardiology, Royal Prince Alfred Hospital, Sydney, New South Wales, Australia.
authors: Rachael
authors: R
authors: Cordina
authors: John
authors: J
authors: Leaney
authors: Mojtaba
authors: M
authors: Golzan
authors: Stuart
authors: S
authors: Grieve
authors: David S
authors: DS
authors: Celermajer
authors: Stuart L
authors: SL
authors: Graham
mesh_terms: D000328:Adult; D003430:Cross-Sectional Studies; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D006801:Humans; D007429:Intraocular Pressure / Q000502:physiology; D008279:Magnetic Resonance Imaging; D008297:Male; D009412:Nerve Fibers / Q000473:pathology*; D010100:Oxygen / Q000097:blood; D012164:Retinal Diseases / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D012165:Retinal Ganglion Cells / Q000473:pathology*; D012171:Retinal Vessels / Q000473:pathology*; D012607:Scotoma / Q000175:diagnosis; D041623:Tomography, Optical Coherence; D014792:Visual Acuity / Q000502:physiology; D014794:Visual Fields / Q000502:physiology
chemical_list: D010100:Oxygen
keywords: cardiovascular disease; retina; retinal pathology; retinal vessel; visual field