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Pulmonary arteriovenous malformations in hereditary haemorrhagic telangiectasia: Correlations between computed tomography findings and cerebral complications

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Abstract

Objectives

Computed tomography (CT) is the modality of choice to characterise pulmonary arteriovenous malformations (PAVMs) in patients with hereditary haemorrhagic telangiectasia (HHT). Our objective was to determine if CT findings were associated with frequency of brain abscess and ischaemic stroke.

Methods

This retrospective study included patients with HHT-related PAVMs. CT results, i.e. PAVM presentation (unique, multiple, disseminated or diffuse), the number of PAVMs and the largest feeding artery size, were correlated to prevalence of ischaemic stroke and brain abscess. All CTs were reviewed in consensus by two radiologists.

Results

Of 170 patients, 73 patients had unique (42.9 %), 49 multiple (28.8 %), 36 disseminated (21.2 %) and 12 diffuse (7.1 %) PAVMs. Fifteen patients presented with brain abscess; 26 patients presented with ischaemic stroke. The number of PAVMs was significantly correlated with brain abscess (11.5 vs. 6.2, respectively; p=0.025). The mean diameter of the largest feeding artery was significantly correlated with ischaemic stroke frequency (4.9 vs. 3.2 mm, respectively; p=0.0098).

Conclusions

The number of PAVMs correlated significantly with risk of brain abscess, and a larger feeding artery significantly with more ischaemic strokes. These findings can lead to a better recognition and management of the PAVMs at risk of cerebral complications.

Key Points

• Chest CT helps clinicians to facilitate appropriate PAVM management strategies.

• Pulmonary arteriovenous malformation CT findings are correlated with risk of cerebral complications.

• Risk of brain abscess is significantly correlated with number of PAVMs.

• Risk of ischaemic stroke is significantly correlated with large feeding artery PAVMs.

• Prevalence of observed of brain abscess and ischaemic stroke is 26 %.

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Abbreviations

ALK1:

Activin receptor-like kinase-1

AVM:

Arteriovenous malformation

CIROCO:

Clinical Investigation and Research for the Rendu–Osler Cohort, national database for HHT in France

CT:

Computed tomography

ENG:

Endoglin

FA:

Feeding artery

HHT:

Hereditary haemorrhagic telangiectasia, Rendu–Osler disease

MIP:

Mode maximum intensity projection on CT

PAVM:

Pulmonary arteriovenous malformation

SD:

Standard deviation

TCE:

Transcatheter embolotherapy

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Funding

The authors state that this work has not received any funding.

Author information

Author notes

  1. J. Etievant and S. Si-mohamed contributed equally as first authors

Authors and Affiliations

  1. Hospices Civils de Lyon, Hôpital Cardiologique Louis Pradel, Département d’Imagerie Cardiaque et Thoracique, Diagnostique et Interventionnelle, 59 Boulevard Pinel, 69500, Bron, France

    Johan Etievant, Salim Si-Mohamed, Nicolas Vinurel, Delphine Gamondes & Didier Revel

  2. Université Claude Bernard Lyon 1, Villeurbanne, France

    Johan Etievant, Salim Si-Mohamed, Nicolas Vinurel, Evelyne Decullier, Vincent Cottin & Didier Revel

  3. Hospices Civils de Lyon, Hôpital Femme-Mère-Enfant, Service de Génétique, Centre de Référence pour la maladie de Rendu-Osler, Lyon, France

    Sophie Dupuis-Girod

  4. Hospices Civils de Lyon, Pôle Information Médicale Évaluation Recherche, Lyon, France

    Evelyne Decullier

  5. Hospices Civils de Lyon, Hôpital Cardiologique Louis Pradel, Service de pneumologie – Centre des Maladies Orphelines Pulmonaires, Lyon, France

    Chahera Khouatra & Vincent Cottin

Authors
  1. Johan Etievant
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  2. Salim Si-Mohamed
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  3. Nicolas Vinurel
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  4. Sophie Dupuis-Girod
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  5. Evelyne Decullier
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  6. Delphine Gamondes
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  7. Chahera Khouatra
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  8. Vincent Cottin
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  9. Didier Revel
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Corresponding author

Correspondence to Salim Si-Mohamed.

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Guarantor

The scientific guarantor of this publication is Professor Didier Revel.

Conflict of interest

The authors of this manuscript declare no relationships with any companies whose products or services may be related to the subject matter of the article.

Statistics and biometry

E. Decullier kindly provided statistical advice for this manuscript.

Informed consent

Written informed consent was obtained from all subjects (patients) in this study.

Ethical approval

Institutional Review Board approval was obtained.

Methodology

• retrospective

• observational

• performed at one institution

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Etievant, J., Si-Mohamed, S., Vinurel, N. et al. Pulmonary arteriovenous malformations in hereditary haemorrhagic telangiectasia: Correlations between computed tomography findings and cerebral complications. Eur Radiol 28, 1338–1344 (2018). https://doi.org/10.1007/s00330-017-5047-x

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  • DOI: https://doi.org/10.1007/s00330-017-5047-x

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