Pulmonary arteriovenous malformations

Moussouttas, Michael; Fayad, Pierre; Rosenblatt, Melvin; Hashimoto, Manabu; Pollak, Jeffrey; Henderson, Katharine J.; T.Y., Z.; White, Robert I.

doi:10.1212/wnl.55.7.959

Cited by 241 publications

(164 citation statements)

References 19 publications

(30 reference statements)

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“…PAVMs are abnormal dilated vascular channels that provide a direct communication between pulmonary arteries and pulmonary veins, and hence a right-to-left shunt ( Figure 1). In cross-sectional studies, approximately one in eight patients with PAVMs experience a clinical stroke (3,4) with imaging revealing a far higher ischaemic burden: in a study performed using 20th century scanners, 34/67 (51%) patients with median age 41 year had evidence of cortical or subcortical infarcts (5).…”

Section: Introductionmentioning

confidence: 99%

Iron deficiency, ischaemic strokes, and right-to-left shunts: From pulmonary arteriovenous malformations to patent foramen ovale?

Shovlin

2014

IRDR

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18.06.14 KB. Ok to add published version, publisher has given written permission.Has the recent identification of iron deficiency as a risk factor for ischaemic stroke in patients with pulmonary arteriovenous malformations (AVMs) unmasked a new paradigm for stroke/infarct pathogenesis? This commentary reviews evidence that spans associations between iron deficiency and ischaemic strokes, iron deficiency enhancement of platelet aggregation in response to serotonin/5HT, settings in which plasma 5HT is elevated, and clinical trial confirmation that 5HT receptor antagonists prevent ischaemic stroke. The critical leap which directs attention away from atherothrombotic events at the neurovascular wall is that ischaemic strokes due to pulmonary AVMs are attributable to compromised pulmonary capillary bed filtration of venous blood. Right-to-left shunting is continuous through pulmonary AVMs, but also occurs intermittently in approximately 30% of the general population with intracardiac shunts such as patent foramen ovale (PFO). The testable hypothesis presented is that paradoxical embolism of venous platelet-based aggregates may constitute part of the causal chain between iron deficiency and ischaemic stroke, not only in the rare disease state of pulmonary AVMs, but also in major subgroups of the general population

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Section: Introductionmentioning

confidence: 99%

Iron deficiency, ischaemic strokes, and right-to-left shunts: From pulmonary arteriovenous malformations to patent foramen ovale?

Shovlin

2014

IRDR

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“…Pulmonary, gastrointestinal and cerebral AVMs are more common than spinal and other visceral AVMs [5,7,21]. Pulmonary and cerebral AVMs can cause complications, such as intracranial bleeding, brain abscess, ischaemic stroke and pulmonary haemorrhage, and screening of at-risk populations has been advocated [23][24][25][26][27][28][29][30][31][32]. Studies in adult patients have demonstrated that significant pulmonary and cerebral AVMs can be detected in asymptomatic patients [5,6,22,[31][32][33][34][35][36][37][38][39], and that preventative treatment can reduce the rate of complications [32].…”

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confidence: 99%

Screening for pulmonary and cerebral arteriovenous malformations in children with hereditary haemorrhagic telangiectasia

Al-Saleh¹,

Mei-Zahav²,

Me³

et al. 2009

European Respiratory Journal

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Hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant disease characterised by vascular dysplasia complicated by visceral arteriovenous malformations (AVMs). To date, the diagnostic yield of screening procedures for pulmonary and cerebral AVMs in children with definite or potential HHT is not well defined. The aim of the present study was to prospectively evaluate the diagnostic yield of a screening protocol for pulmonary and cerebral AVMs in children with either a definite or potential HHT diagnosis.All children referred for evaluation for HHT between 1996 and 2008 were included in the present analysis. Screening tests for AVMs included chest computed tomography and brain magnetic resonance imaging.61 children with a definite clinical and/or genetic diagnosis of HHT were asymptomatic for visceral AVMs at their first baseline assessment (mean¡SD age 8.7¡4.7 yrs; range 0-17.0 yrs). Of these, 15 (25%) had pulmonary and/or cerebral AVMs diagnosed on initial screening tests. Pulmonary AVMs predominated in paediatric HHT patients (14 out of 15 patients) and were found in eight children aged ,10 yrs. 55 children had a potential HHT diagnosis as they fulfilled only one or two HHT clinical diagnostic criteria and did not have a confirmatory genetic diagnosis (age 10.9¡4.8 yrs; range 0-17.9 yrs). None of these children had pulmonary or cerebral AVMs on initial screening tests.The present data suggest that children with a definite HHT diagnosis have a high frequency of pulmonary AVMs even when clinically asymptomatic. In contrast, no AVMs were observed in children not fulfilling HHT diagnostic criteria. Genetic testing appears to be useful in defining an at-risk group for pulmonary AVMs in childhood.

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“…Additionally, a relationship between right to left shunts and migraine with aura, symptoms of which may mimic acute stroke, has been previously described in the HHT population in the literature [3] . Among patients with multiple PAVMs, the risk of neurologic complications appears to be increased further [4] . However, early detection and embolization of PAVMs can reduce neurologic complications in individuals with HHT [1,5] .…”

Section: Discussionmentioning

confidence: 99%

“…Chest radiography, arterial blood gas measurement with alveolar-arterial gradient assessment when the patient is breathing 100% inspired oxygen, TTCE, and radionucleotide perfusion scanning have all been used to screen for PAVMs [4][5][6][7] . Conventional pulmonary angiography or chest CT is considered the gold standard for detection of PAVMs [1] .…”

Section: Discussionmentioning

confidence: 99%

Transthoracic contrast echocardiography for detection of pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: How can the process be improved for patient safety?

Fan¹,

Choy²,

Shanks³

et al. 2014

IJDI

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Introduction: Patients with hereditary hemorrhagic telangiectasia (HHT) are at risk for pulmonary arteriovenous malformations (PAVMs). Extracardiac right-to-left shunts due to PAVMs can lead to systemic emboli; clinical sequelae including strokes and brain abscesses. Regular screening is required, with appropriate medical/interventional management to alleviate morbidity and mortality from PAVMs. International HHT guidelines recommend standardized transthoracic contrast echocardiogram (TTCE) with agitated saline (in an HHT Foundation-accredited laboratory) as the initial screening test, and, when needed, non-contrast computerized tomography of the chest (CT Chest) to follow up on a positive TTCE.Case presentation: A 40-year-old woman was seen for evaluation of HHT and referred for TTCE as part of her PAVM screen. Following administration of agitated saline, she developed a headache, visual changes in her right eye, and right arm weakness, followed by posturing and stiffness in her right arm. Her symptoms resolved after five minutes, consistent with a transient ischemic attack (TIA).Clinical impact: Protocols at our institution were amended following this index case and echocardiography laboratory protocols were adjusted to use a maximum of 0.1 cc air to 9.9 cc saline for TTCE studies in the HHT population. Only the attending echocardiologist who is familiar with possible systemic air embolism during bubble contrast echocardiography in patients with HHT should perform agitation and infusion of contrast and should directly review the images for appropriate visualization during the exam for this test to be appropriately interpreted. Paradoxical emboli due to right-to-left shunt are important to recognize. Best practice for PAVM screening in HHT patients includes use of standardized protocols for the performance and reporting of TTCE in the evaluation of pulmonary shunts.

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Pulmonary arteriovenous malformations

Abstract: There is a strong association between single PAVM and various neurologic manifestations. The prevalence is greater for patients with multiple PAVM, suggesting increased predisposition for paradoxical embolization with a greater number of malformations.

Cited by 241 publications

References 19 publications

Iron deficiency, ischaemic strokes, and right-to-left shunts: From pulmonary arteriovenous malformations to patent foramen ovale?

Iron deficiency, ischaemic strokes, and right-to-left shunts: From pulmonary arteriovenous malformations to patent foramen ovale?

Screening for pulmonary and cerebral arteriovenous malformations in children with hereditary haemorrhagic telangiectasia

Transthoracic contrast echocardiography for detection of pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: How can the process be improved for patient safety?

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