title: Familial primary pulmonary hypertension and associated ocular findings.
abstract: BACKGROUND
  Familial primary pulmonary hypertension (PPH) is a rare, fatal, autosomal dominant disease that results in right heart failure from idiopathic obliteration of the pulmonary arteries. Patients develop stagnation of venous blood flow and elevated venous pressure.
  
  
  METHODS
  The authors retrospectively reviewed the clinical records of three patients diagnosed with PPH that was confirmed on the basis of physical examination, chest X-ray, electrocardiogram, and echocardiogram. Cardiac catheterization excluded cardiac shunt and other secondary causes of pulmonary hypertension.
  
  
  RESULTS
  Two patients presented with a clinical picture resembling venous stasis retinopathy, and one with bilateral choroidal detachments. Two patients had delayed choroidal filling on fluorescein angiography, which was confirmed in one patient with indocyanine green videoangiography.
  
  
  CONCLUSIONS
  Elevated venous pressure found in PPH is responsible for the delayed choroidal perfusion and the reduced venous blood outflow. This explains the clinical findings of venous stasis retinopathy and choroidal detachments seen in these patients.
authors.affiliation: Chester County Eye Care Associates, P.C., West Chester, Pennsylvania, USA.
authors.forename: B R
authors.initials: BR
authors.lastname: Saran
authors.forename: A J
authors.initials: AJ
authors.lastname: Brucker
authors.forename: F
authors.initials: F
authors.lastname: Bandello
authors.forename: C
authors.initials: C
authors.lastname: Verougstraete
mesh_terms: D000328:Adult; D001783:Blood Flow Velocity; D002302:Cardiac Output; D002829:Choroid / Q000098:blood supply; D015862:Choroid Diseases / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D004452:Echocardiography; D004562:Electrocardiography; D005260:Female; D005451:Fluorescein Angiography; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000175:diagnosis / Q000235:genetics*; D007208:Indocyanine Green; D008297:Male; D013902:Radiography, Thoracic; D012170:Retinal Vein Occlusion / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D012189:Retrospective Studies; D014690:Venous Pressure; D014792:Visual Acuity
chemical_list: D007208:Indocyanine Green