title: A Survey of Pulmonary Arteriovenous Malformation Screening, Management, and Follow-Up in Hereditary Hemorrhagic Telangiectasia Centers of Excellence.
abstract: OBJECTIVE
  To determine current practice regarding screening, management, and follow-up of pulmonary arteriovenous malformations (PAVMs), particularly those with feeding arteries <3 mm in diameter, in those patients with hereditary hemorrhagic telangiectasia (HHT) at HHT Centers of Excellence.
  
  
  METHODS
  A 32-question survey focusing on PAVM screening, management, and follow-up was sent to 147 participants at HHT Centers worldwide.
  
  
  RESULTS
  Thirty-three responses were received (22%); 60% from interventional radiologists and 20% from pulmonologists; 67% from the USA, 21% European, and 6% Asian. In HHT patients with initial negative screening echocardiogram, 60% recommended repeat screening contrast echocardiogram in 5 years, while 15% felt that no additional screening was necessary in these patients. Fifty-nine percentage reported never having seen a patient with initial negative contrast echocardiogram become positive. In patients with grade 1 echocardiograms, 41% recommended contrast-enhanced CT as the next step in management, 22% recommended non-contrast CT, and 22 and 3% recommended repeat echocardiogram in 5 and 10 years, respectively. In patients with grade 1 echocardiograms but no visible PAVMs, opinions regarding the use of bubble filters were evenly divided, while 79% recommended prophylactic antibiotics prior to invasive procedures. Eighty percentages of respondents treat PAVMs with feeding artery less than 3 mm, and 52% treat feeders less than 2 mm. Regarding follow-up, 71% reported seeing PAVM recurrence at the site of a previously completely treated PAVM.
  
  
  CONCLUSIONS
  There is inconsistency in practice regarding PAVM screening, management, and follow-up, suggesting a need for further research and guideline development.
authors.affiliation: Division of Interventional Radiology, Department of Radiology, Perelman School of Medicine, Hospital of the University of Pennsylvania, 1 Silverstein, 3400 Spruce Street, Philadelphia, PA, 19104, USA.|Division of Vascular and Interventional Radiology, Department of Radiology, University of Michigan Medical Center, 1500 East Medical Center Drive, Ann Arbor, MI, 48109, USA.
authors.forename: Jeffrey Forris Beecham
authors.initials: JFB
authors.lastname: Chick
authors.affiliation: Division of Interventional Radiology, Department of Radiology, Perelman School of Medicine, Hospital of the University of Pennsylvania, 1 Silverstein, 3400 Spruce Street, Philadelphia, PA, 19104, USA.
authors.forename: Shilpa N
authors.initials: SN
authors.lastname: Reddy
authors.affiliation: Division of Translational Medicine and Human Genetics, Department of Medicine, Perelman School of Medicine, Hospital of the University of Pennsylvania, 1 Silverstein, 3400 Spruce Street, Philadelphia, PA, 19104, USA.|Hereditary Hemorrhagic Telangiectasia Center of Excellence, Perelman School of Medicine, Hospital of the University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA, 19104, USA.
authors.forename: Reed E
authors.initials: RE
authors.lastname: Pyeritz
authors.affiliation: Division of Interventional Radiology, Department of Radiology, Perelman School of Medicine, Hospital of the University of Pennsylvania, 1 Silverstein, 3400 Spruce Street, Philadelphia, PA, 19104, USA. scott.trerotola@uphs.upenn.edu.|Hereditary Hemorrhagic Telangiectasia Center of Excellence, Perelman School of Medicine, Hospital of the University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA, 19104, USA. scott.trerotola@uphs.upenn.edu.
authors.forename: Scott O
authors.initials: SO
authors.lastname: Trerotola
mesh_terms: D000328:Adult; D001164:Arteriovenous Fistula / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy*; D003287:Contrast Media; D003430:Cross-Sectional Studies; D004452:Echocardiography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008403:Mass Screening; D008875:Middle Aged; D011651:Pulmonary Artery / Q000002:abnormalities*; D011667:Pulmonary Veins / Q000002:abnormalities*; D011795:Surveys and Questionnaires; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy*
chemical_list: D003287:Contrast Media
keywords: Hereditary hemorrhagic telangiectasia; Interventional radiology; Medical genetics; Pulmonary arteriovenous malformation