Unusual Complications After Embolization of a Pulmonary Arteriovenous Malformation

Haitjema, T.; Berg, J. M. ten; Overtoom, T. T. C.; Ernst, J. M. P. G.; Westermann, C. J. J.

doi:10.1378/chest.109.5.1401

Cited by 49 publications

(35 citation statements)

References 7 publications

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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%

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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attractive experimental therapies promoted in high profile publications when guidelines and reviews are quickly superseded.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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Section: Overview Of Hhtmentioning

confidence: 99%

“…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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Hereditary haemorrhagic telangiectasia: Pathophysiology, diagnosis and treatment

2010

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“…In three out of the four reported cases [9,17,32], each selected from larger series, Ppa increased postembolisation, while in the fourth it was unchanged [11]. There is also a report of worsening PH after surgical resection of a pulmonary AVM [43].…”

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confidence: 93%

“…Typically recognised by nosebleeds, mucocutaneous telangiectasia and visceral AVMs [21], HHT may be associated with PH [9,[22][23][24][25][26][27][28][29][30]. The secondary causes of PH in HHT are diverse, as they are in the normal population [31], but PH particularly occurs either as a true pulmonary arterial hypertension (PAH) phenotype [9,22,[28][29][30] or in the context of high output cardiac failure secondary to hepatic AVMs, when PH may be reversible after hepatic AVM treatment [32]. The frequencies of PAH and hepatic AVMs differ with HHT genotype: HHT is caused by mutations in at least five genes, including endoglin (HHT type 1) and ALK-1 (HHT type 2), with pulmonary AVMs most common in HHT type 1 [33].…”

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confidence: 99%

“…Out of .700 reported pulmonary AVM embolisations [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][34][35][36][37][38][39][40][41][42], data on Ppa measurements pre-and post-embolisation are scarce [9,11,17,32]. In three out of the four reported cases [9,17,32], each selected from larger series, Ppa increased postembolisation, while in the fourth it was unchanged [11].…”

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

Shovlin¹,

Tighe²,

Davies³

et al. 2008

Eur Respir J

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Increasing evidence supports the use of embolisation to treat pulmonary arteriovenous malformations (AVMs). Most pulmonary AVM patients have hereditary haemorrhagic telangiectasia (HHT), a condition that may be associated with pulmonary hypertension.The current authors tested whether pulmonary AVM embolisation increases pulmonary artery pressure (Ppa) in patients without baseline severe pulmonary hypertension. Ppa was measured at the time of pulmonary AVM embolisation in 143 individuals, 131 (92%) of whom had underlying HHT. Angiography/embolisation was not performed in four individuals with severe pulmonary hypertension, whose systemic arterial oxygen saturation exceeded levels usually associated with dyspnoea in pulmonary AVM patients.In 143 patients undergoing pulmonary AVM embolisation, Ppa was significantly correlated with age, with the most significant increase occurring in the upper quartile (aged .58 yrs). In 43 patients with repeated measurements, there was no significant increase in Ppa as a result of embolisation. In half, embolisation led to a fall in Ppa. The maximum rise in mean Ppa was 8 mmHg: balloon test occlusion was performed in one of these individuals, and did not predict the subsequent rise in Ppa following definitive embolisation of the pulmonary AVMs.In the present series of patients, which excluded those with severe pulmonary hypertension, pulmonary artery pressure was not increased significantly by pulmonary arteriovenous malformation embolisation.KEYWORDS: Brain abscess, hypoxaemia, nosebleeds, oxygen saturation, right-to-left shunt, stroke D oes embolisation of pulmonary arteriovenous malformations (AVMs) precipitate pulmonary hypertension (PH)? The reason this question is important is that for individuals with pulmonary AVMs, embolisation is an effective means of reducing lifetime risks of paradoxical embolic stroke and brain abscess [1,2], improving oxygenation [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17] and treating pulmonary AVM-related haemoptysis [18,19]. Conversely, embolisation may be expected to elevate pulmonary artery pressure (Ppa), since pulmonary AVMs are abnormal dilated vessels between pulmonary arteries and veins that provide low resistance pathways for pulmonary blood flow [20].The question of whether pulmonary AVM embolisation increases Ppa is particularly pertinent, since most individuals with pulmonary AVMs have underlying hereditary haemorrhagic telangiectasia (HHT). Typically recognised by nosebleeds, mucocutaneous telangiectasia and visceral AVMs [21], HHT may be associated with PH [9,[22][23][24][25][26][27][28][29][30]. The secondary causes of PH in HHT are diverse, as they are in the normal population [31], but PH particularly occurs either as a true pulmonary arterial hypertension (PAH) phenotype [9,22,[28][29][30] or in the context of high output cardiac failure secondary to hepatic AVMs, when PH may be reversible after hepatic AVM treatment [32]. The frequencies of PAH and hepatic AVMs differ with HHT genotype: HHT is caused by mutatio...

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Pulmonary Arteriovenous Malformations and Hereditary Hemorrhagic Telangiectasia: Embolotherapy Using Balloons and Coils-Reply

Haitjema¹

1996

Arch Intern Med

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systemic circulation," thereby producing serious occlusion of a systemic artery. Detachable silicone balloons and stainless steel coils with Dacron fibers have been used for 17 years by interventional radiologists to close PAVMs, and my colleagues and I believe that both have an important role in providing safe and efficacious treatment.2 We have not observed late migration of a detachable silicone balloon or a coil once they are securely in place in the artery leading to a PAVM in any of the more than 250 patients whom we have treated. We do agree that balloons, like coils, can dislocate at the time of place¬ ment if they are not properly secured into the artery lead¬ ing to the PAVM. If migration of the occlusion device oc¬ curs at the time of placement, it will enter the systemic circulation with potentially serious consequences.2The experience with detachable silicone balloons, both experimentally and clinically, demonstrates that when they deflate, and approximately 10% do over the course of the first 1 to 2 years, the deflated shell will remain attached to the wall of the artery or embedded within an organized thrombus.3*4 Experimentally, balloons deflating even af¬ ter as short a time as 2 weeks after placement are also trapped within an organized thrombus.3 The late defla¬ tion that Haitjema and colleagues reference in the series of patients described by Puskas et aL involved 1 patient with a deflation of f balloon documented at 4 months af¬ ter placement. There was no clinical evidence mentioned to indicate that the balloon or its deflated shell migrated into the systemic circulation. The angiograms from this patient were not shown. It is quite possible that, rather than recanalization, this PAVM recurred because an accessory vessel had enlarged at the time of restudy 5 years later, ac¬ counting for the continued patency.Detachable silicone balloons filled with isosmotic contrast media produce excellent cross-sectional occlu¬ sion of PAVMs.4*6*7 My colleagues and I believe that there are advantages and disadvantages to the use of both coils and balloons, depending on the anatomical situation, and that radiologists treating PAVMs should have access and familiarity with both types of occlusion devices. Robert I. White, Jr, MD New Haven, Conn 1. Haitjema T, Westermann CJJ, Overtoom TTC, et al. Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease): new insights in pathogenesis, complications, and treatment. Arch Intern Med. 1996;156:714-719. 2. White RI, Nyhan AL, Terry P, Buescher PC, et al. Pulmonary arteriovenous malformations: techniques and long-term outcome of embolotherapy. Radiology. 1988;169:633-669. 3. Kaufman SL, Strandberg JV, Barth KH, Gross GS, White RI. Therapeutic embolization with detachable Silastic balloon: long-term effects in swine. Invest Radiol. 1979;14:156-161. 4. Pollak JS, Egglin TK, Rosenblatt MM, Dickey KW, White RI. Clinical results of transvenous systemic embolotherapy with a neuroradiological detachable balloon. Radiology. 1994;191:477-482. 5. Puskas JD, Allen MS, M...

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Unusual Complications After Embolization of a Pulmonary Arteriovenous Malformation

Cited by 49 publications

References 7 publications

Hereditary haemorrhagic telangiectasia: Pathophysiology, diagnosis and treatment

Hereditary haemorrhagic telangiectasia: Pathophysiology, diagnosis and treatment

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

Pulmonary Arteriovenous Malformations and Hereditary Hemorrhagic Telangiectasia: Embolotherapy Using Balloons and Coils-Reply

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