Jeffrey Forris Beecham Chick1,2, Shilpa N Reddy1, Reed E Pyeritz3,4, Scott O Trerotola5,6. 1. 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. 2. Division of Vascular and Interventional Radiology, Department of Radiology, University of Michigan Medical Center, 1500 East Medical Center Drive, Ann Arbor, MI, 48109, USA. 3. 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. 4. Hereditary Hemorrhagic Telangiectasia Center of Excellence, Perelman School of Medicine, Hospital of the University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA, 19104, USA. 5. 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. 6. 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.
Abstract
PURPOSE: 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. MATERIALS AND 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. CONCLUSION: There is inconsistency in practice regarding PAVM screening, management, and follow-up, suggesting a need for further research and guideline development.
PURPOSE: 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. MATERIALS AND 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 HHTpatients 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. CONCLUSION: There is inconsistency in practice regarding PAVM screening, management, and follow-up, suggesting a need for further research and guideline development.
Authors: Joan D Beckman; Quefeng Li; Samuel T Hester; Ofri Leitner; Karen L Smith; Raj S Kasthuri Journal: Orphanet J Rare Dis Date: 2020-07-13 Impact factor: 4.123
Authors: William Salibe-Filho; Bruna Mamprim Piloto; Ellen Pierre de Oliveira; Marcela Araújo Castro; Breno Boueri Affonso; Joaquim Maurício da Motta-Leal-Filho; Edgar Bortolini; Mário Terra-Filho Journal: J Bras Pneumol Date: 2019-06-19 Impact factor: 2.624