Embolisation of pulmonary arteriovenous malformations: no consistent effect on pulmonary artery pressure

Shovlin, CL; Tighe, Hannah; Davies, Rachel J.; Gibbs, J. S. R.; Jackson, James E.

doi:10.1183/09031936.00126207

Cited by 70 publications

(81 citation statements)

References 51 publications

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“…Routine screening for pulmonary AVMs has been advocated in the adult population [24-27, 31, 36] as embolisation of pulmonary AVMs is safe and reduces the risk of stroke and brain abscess, as shown in interventional studies [28,30,32,55]. In addition, embolisation therapy has been shown to be safe and effective in children [44].…”

Section: Discussionmentioning

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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Section: Discussionmentioning

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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“…2 The spectrum of disease within the HHT umbrella has extended beyond the telangiectatic/AVM HHT pathology delineated by the Curaçao criteria. 2 More recently recognised features include pulmonary arterial hypertension 10 ; juvenile polyposis 11 ; pulmonary hypertension in the context of high output cardiac failure secondary to hepatic AVMs, when PH may be reversible after hepatic AVM treatment [12][13][14][15][16] ; a prothrombotic state associated with elevated plasma levels of factor VIII 17 , and potential immune dysfunction. 18 Three of the genes mutated in HHT have been identified: endoglin (resulting in HHT1, OMIM #187300) 19 ; ACRVL1/ALK1; (resulting in HHT2, OMIM#600376) 20 , and more rarely, SMAD4 (mutated in HHT in association with juvenile polyposis, JPHT OMIM #175050) 11 .…”

Section: Overview Of Hhtmentioning

confidence: 99%

“…Technical improvements in embolisation treatment devices (Amplatzer devices 59,60 ). Further data on post embolization systemic arterial supply without haemoptysis 61 ; Embolization with preexisting pulmonary hypertension 16,62 ). Dental issues AHA 63 and NICE Guidance 64 , but reconsidered and Abs still recommended 65 .…”

Section: Acknowledgmentsmentioning

confidence: 99%

“…For example, many HHT patients understood that they had a high chance Blood Reviews _ HHT 2010_ Shovlin 6 of developing an aggressive form of pulmonary hypertension. While pulmonary arterial hypertension does affect a subgroup of HHT patients, the real risk is probably closer to 1-2%, 16,101 and is genotype-dependant. 102 Thirdly, the majority of affected individuals still remain undiagnosed: In 121 (59%) of 205 consecutive individuals with pulmonary AVMs and HHT reviewed at one UK institution, the diagnosis of HHT had not been made previously.…”

mentioning

confidence: 98%

“…HHT2 patients are also at higher risk of post capillary pulmonary hypertension associated with hepatic AVMs. [12][13][14][15][16] Blood Reviews _ HHT 2010_ Shovlin 11 …”

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confidence: 99%

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Hereditary haemorrhagic telangiectasia: Pathophysiology, diagnosis and treatment

2010

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Hereditary haemorrhagic telangiectasia, inherited as an autosomal dominant trait, affects approximately 1 in 5,000 people. The abnormal vascular structures in HHT result from mutations in genes (most commonly endoglin or ACVRL1) whose protein products influence TGF-ß superfamily signalling in vascular endothelial cells. The cellular mechanisms underlying the generation of HHT telangiectasia and arteriovenous malformations are being unravelled, with recent data focussing on a defective response to angiogenic stimuli in particular settings. For affected individuals, there is often substantial morbidity due to sustained and repeated haemorrhages from telangiectasia in the nose and gut. Particular haematological clinical challenges include the management of severe iron deficiency anaemia; handling the intricate balance of antiplatelet or anticoagulants for HHT patients in whom there are often compelling clinical reasons to use such agents; and evaluation of apparently attractive experimental therapies promoted in high profile publications when guidelines and reviews are quickly superseded. There is also a need for sound screening programmes for silent arteriovenous malformations. These occur commonly in the pulmonary, cerebral, and hepatic circulations, may haemorrhage, but predominantly result in more complex pathophysiology due to consequences of defective endothelium, or shunts that bypass specific capillary beds. This review will focus on the new evidence and concepts in this complex and fascinating condition, placing these in context for both clinicians and scientists, with a particular emphasis on haematological settings. Blood Reviews _ HHT 2010_ Shovlin 3 Overview of HHTHHT, also known as Osler Weber Rendu syndrome [1][2][3] , is one of the most common disorders to be inherited as an autosomal dominant trait. Careful epidemiological studies reveal that it affects approximately 1 in 5,000 individuals, 4,5 with regional differences, 6 and isolated communities displaying higher prevalences due to founder effects. 7 8 HHT was first described as a familial disease characterised by severe recurrent nasal and gastrointestinal bleeding with associated anaemia, and visible dilated blood vessels (telangiectasia) on the lips and finger tips. The majority of HHT patients are also affected by larger arteriovenous malformations (AVMs) in the pulmonary, hepatic, cerebral, pancreatic, spinal and other circulations. 1,2,9 These features, presented in more detail within Table 1, are used as criteria to diagnose HHT. 2 The spectrum of disease within the HHT umbrella has extended beyond the telangiectatic/AVM HHT pathology delineated by the Curaçao criteria. 2 More recently recognised features include pulmonary arterial hypertension 10 ; juvenile polyposis 11 ; pulmonary hypertension in the context of high output cardiac failure secondary to hepatic AVMs, when PH may be reversible after hepatic AVM treatment [12][13][14][15][16] ; a prothrombotic state associated with elevated plasma levels of factor VIII 17 , and poten...

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Embolisation therapy for pulmonary arteriovenous malformations

Hsu

Kwan

Thompson³

et al. 2010

Cochrane Database of Systematic Reviews

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Background Pulmonary arteriovenous malformations are abnormal direct connections between the pulmonary artery and pulmonary vein which result in a right-to-left shunt. They are associated with substantial morbidity and mortality mainly from the effects of paradoxical emboli. Potential complications include stroke, cerebral abscess, pulmonary haemorrhage and hypoxaemia. Embolisation therapy is a form of treatment based on the occlusion of the feeding arteries to a pulmonary arteriovenous malformation and can prevent many of these debilitating and life-threatening complications. Objectives To determine the efficacy and safety of embolisation therapy in people with pulmonary arteriovenous malformations including a comparison with surgical resection and different embolisation devices. Search methods We searched the Cystic Fibrosis and Genetic Disorders Group's Trials Registers (last searched 07 September 2009). We also searched the following databases: the Australian New Zealand Clinical Trials Registry; ClinicalTrials.gov; International Standard Randomised Controlled Trial Number Register; International Clinical Trials Registry Platform Search Portal (last searched 22 November 2009). We checked cross-references and searched references from review articles. Finally, we contacted manufacturers and specialised centres for unpublished and ongoing trials. Selection criteria Trials in which individuals with pulmonary arteriovenous malformations were randomly allocated to embolisation therapy compared to no treatment, surgical resection or a different embolisation device. Studies identified for potential inclusion were independently assessed for eligibility by two authors, with excluded studies further checked by a third author. Data collection and analysis No trials were identified. As this was the case, no analysis was performed. Main results There were no randomised controlled trials identified.

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Embolisation of pulmonary arteriovenous malformations: no consistent effect on pulmonary artery pressure

Cited by 70 publications

References 51 publications

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

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

Hereditary haemorrhagic telangiectasia: Pathophysiology, diagnosis and treatment

Embolisation therapy for pulmonary arteriovenous malformations

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