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A Survey of Pulmonary Arteriovenous Malformation Screening, Management, and Follow-Up in Hereditary Hemorrhagic Telangiectasia Centers of Excellence

  • Clinical Investigation
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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.

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Abbreviations

PAVMs:

Pulmonary arteriovenous malformations

HHT:

Hereditary hemorrhagic telangiectasia

CT:

Computed tomography

MRI:

Magnetic resonance imaging

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Author notes

    Authors and Affiliations

    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

      Jeffrey Forris Beecham Chick, Shilpa N. Reddy & Scott O. Trerotola

    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

      Jeffrey Forris Beecham Chick

    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

      Reed E. Pyeritz

    4. Hereditary Hemorrhagic Telangiectasia Center of Excellence, Perelman School of Medicine, Hospital of the University of Pennsylvania, 3400 Spruce Street, Philadelphia, PA, 19104, USA

      Reed E. Pyeritz & Scott O. Trerotola

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    1. Jeffrey Forris Beecham Chick
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    2. Shilpa N. Reddy
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    Correspondence to Scott O. Trerotola.

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    The authors whose names are listed immediately below certify that they have no affiliations with or involvement in any organization or entity with any financial interest (such as honoraria; educational grants; participation in speakers’ bureaus; membership, employment, consultancies, stock ownership, or other equity interest; and expert testimony or patent-licensing arrangements), or non-financial interest (such as personal or professional relationships, affiliations, knowledge, or beliefs) in the subject matter or materials discussed in this manuscript.

    Human and Animal Rights

    All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

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    Informed consent was obtained from all individual participants included in the study.

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    Jeffrey Forris Beecham Chick, Shilpa N. Reddy, Reed E. Pyeritz and Scott O. Trerotola have read and contributed to this manuscript.

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    Chick, J.F.B., Reddy, S.N., Pyeritz, R.E. et al. A Survey of Pulmonary Arteriovenous Malformation Screening, Management, and Follow-Up in Hereditary Hemorrhagic Telangiectasia Centers of Excellence. Cardiovasc Intervent Radiol 40, 1003–1009 (2017). https://doi.org/10.1007/s00270-017-1604-6

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