CIRSE Standards of Practice on Diagnosis and Treatment of Pulmonary Arteriovenous Malformations

Müller‐Hülsbeck, Stefan; Marques, Leonardo; Maleux, Geert; Osuga, Keigo; Pelage, Jean-Pierre; Wohlgemuth, Walter A.; Andersen, Poul Erik

doi:10.1007/s00270-019-02396-2

Cited by 43 publications

(65 citation statements)

References 46 publications

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“…One study reports a high success rate treating recanalized PAVMs using coils in conjunction with Amplatzer vascular plugs [61]. In either case, dense packing and complete stasis in the targeted AVM should be the desired endpoint [16].…”

Section: Persistent Pavmsmentioning

confidence: 99%

“…Conversely, coils with too large of a diameter may lead to inadequate packing, or rarely complications such as vessel rupture [92]. For pushable coils, 20-30% coil oversizing relative to feeding vessel diameter is recommended [16,92]. With newer, longer detachable coil designs, coil size can be closely matched to the vessel diameter, as the longer coils provide more vessel wall contact to prevent migration.…”

Section: Embolic Devicesmentioning

confidence: 99%

“…One study found AVPs to be very effective in treatment of 24 large PAVMs with feeding artery diameter ≥8 mm, reporting no persistence, migration, or complications in follow-up [102]. Studies show that vascular plugs are less likely to recanalize compared to coils, and that vascular plug alone or in combination with coils might be a better primary option for PAVM embolization when technically feasible [16,103,104]. A 2012 study achieved high rates of technical success treating complex PAVMs by first performing venous sac embolization with detachable coils, followed by occlusion of the large feeding arteries using AVPs [105].…”

Section: Embolic Devicesmentioning

confidence: 99%

“…Lastly, some authors suggest the use of time-resolved magnetic resonance imaging (MRI) for follow-up, particularly in patients treated with coils as there may be less induced metallic artifact with this modality [3,16,115,116]. A recent pilot study compared the use of ferumoxytol-enhanced MR angiography (MRA) to CT angiography (CTA) for PAVM detection [81].…”

Section: Follow-upmentioning

confidence: 99%

“…The risk of brain abscess is significantly correlated with number of PAVMs, and larger feeding artery size is significantly correlated with risk of ischemic stroke [15]. Current indications for treatment of PAVM include any (solitary or multiple) PAVM with feeding artery diameter ≥2-3 mm, measurable increase in PAVM size, paradoxical emboli, symptomatic hypoxemia, or any of the other aforementioned serious complications [16].…”

Section: Introductionmentioning

confidence: 99%

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Approach to Pulmonary Arteriovenous Malformations: A Comprehensive Update

Majumdar

McWilliams

2020

JCM

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Pulmonary arteriovenous malformations (PAVMs) are abnormal direct vascular communications between pulmonary arteries and veins which create high-flow right-to-left shunts. They are most frequently congenital, usually in the setting of hereditary hemorrhagic telangiectasia (HHT). PAVMs may be asymptomatic or present with a wide variety of clinical manifestations such as dyspnea, hypoxemia, or chest pain. Even when asymptomatic, presence of PAVMs increases patients’ risk of serious, potentially preventable complications including stroke or brain abscess. Transcatheter embolotherapy is considered the gold standard for treatment of PAVMs. Though previous guidelines have been published regarding the management of PAVMs, several aspects of PAVM screening and management remain debated among the experts, suggesting the need for thorough reexamination of the current literature. The authors of this review present an updated approach to the diagnostic workup and management of PAVMs, with an emphasis on areas of controversy, based on the latest literature and our institutional experience.

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Section: Persistent Pavmsmentioning

confidence: 99%

Section: Embolic Devicesmentioning

confidence: 99%

Section: Embolic Devicesmentioning

confidence: 99%

Section: Follow-upmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Approach to Pulmonary Arteriovenous Malformations: A Comprehensive Update

Majumdar

McWilliams

2020

JCM

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Unsupervised machine learning algorithms identify expected haemorrhage relationships but define unexplained coagulation profiles mapping to thrombotic phenotypes in hereditary haemorrhagic telangiectasia

Mukhtar

Shovlin

2023

eJHaem

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Hereditary haemorrhagic telangiectasia (HHT) can result in challenging anaemia and thrombosis phenotypes. Clinical presentations of HHT vary for relatives with identical casual mutations, suggesting other factors may modify severity. To examine objectively, we developed unsupervised machine learning algorithms to test whether haematological data at presentation could be categorised into sub‐groupings and fitted to known biological factors. With ethical approval, we examined 10 complete blood count (CBC) variables, four iron index variables, four coagulation variables and eight iron/coagulation indices combined from 336 genotyped HHT patients (40% male, 60% female, 86.5% not using iron supplementation) at a single centre. T‐SNE unsupervised, dimension reduction, machine learning algorithms assigned each high‐dimensional datapoint to a location in a two‐dimensional plane. k‐Means clustering algorithms grouped into profiles, enabling visualisation and inter‐profile comparisons of patients’ clinical and genetic features. The unsupervised machine learning algorithms using t‐SNE and k‐Means identified two distinct CBC profiles, two iron profiles, four clotting profiles and three combined profiles. Validating the methodology, profiles for CBC or iron indices fitted expected patterns for haemorrhage. Distinct coagulation profiles displayed no association with age, sex, C‐reactive protein, pulmonary arteriovenous malformations (AVMs), ENG/ACVRL1 genotype or epistaxis severity. The most distinct profiles were from t‐SNE/k‐Means analyses of combined iron‐coagulation indices and mapped to three risk states – for venous thromboembolism in HHT; for ischaemic stroke attributed to paradoxical emboli through pulmonary AVMs in HHT; and for cerebral abscess attributed to odontogenic bacteremias in immunocompetent HHT patients with right‐to‐left shunting through pulmonary AVMs. In conclusion, unsupervised machine learning algorithms categorise HHT haematological indices into distinct, clinically relevant profiles which are independent of age, sex or HHT genotype. Further evaluation may inform prophylaxis and management for HHT patients’ haemorrhagic and thrombotic phenotypes.

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Pulmonary Angiography: Arteriovenous Malformation and Pseudoaneurysm

Luhar,

Pollak

2024

IR Playbook

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CIRSE Standards of Practice on Diagnosis and Treatment of Pulmonary Arteriovenous Malformations

Cited by 43 publications

References 46 publications

Approach to Pulmonary Arteriovenous Malformations: A Comprehensive Update

Approach to Pulmonary Arteriovenous Malformations: A Comprehensive Update

Unsupervised machine learning algorithms identify expected haemorrhage relationships but define unexplained coagulation profiles mapping to thrombotic phenotypes in hereditary haemorrhagic telangiectasia

Pulmonary Angiography: Arteriovenous Malformation and Pseudoaneurysm

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