Life-Threatening Pulmonary Hemorrhage With Pulmonary Arteriovenous Malformations and Hereditary Hemorrhagic Telangiectasia

Ference, Brian А.; Shannon, Timothy; White, Robert I.; Zawin, Marlene; Burdge, Catherine M.

doi:10.1378/chest.106.5.1387

Cited by 216 publications

(122 citation statements)

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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%

“…Whether this is due to a lower rate of pulmonary and cerebral manifestations in children is unclear. To date, frequency estimates of pulmonary and cerebral AVMs are based on case reports and small series [8,24,26,27,[40][41][42][43][44][45][46][47][48][49][50][51], and the diagnostic yield of initial diagnostic screening procedures in asymptomatic children has not been systematically evaluated.…”

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confidence: 99%

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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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confidence: 99%

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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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“…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].…”

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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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confidence: 99%

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

Shovlin¹,

Tighe²,

Davies³

et al. 2008

European Respiratory Journal

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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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“…Another risk, aside from the increased risk of rupture presenting with massive hemoptysis and/or hemothorax, includes significant right to left shunt resulting in hypoxemia, heart failure, and the potential passage of emboli across the shunt into the systemic circulation causing complications, such as stroke and cerebral or systemic abscesses. 8,[18][19][20] In Shovlin's series of 262 pregnancies, 1% were associated with a major PAVM bleed, 1.2% were associated with stroke (though not all HHT related), and 1% were associated with maternal death. A prior diagnosis of HHT or PAVM was associated with improved survival in the event of a major complication in pregnancy.…”

Section: Pulmonary Features Of Hhtmentioning

confidence: 99%

Anesthetic implications for the parturient with hereditary hemorrhagic telangiectasia

Lomax

Edgcombe

2009

Can J Anesth/J Can Anesth

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Purpose To review the effects of hereditary hemorrhagic telangiectasia (HHT) in the parturient and the anesthetic management of such patients during pregnancy and delivery. Source A literature search was performed using Medline and EMBASE databases. Bibliographies of retrieved articles were searched for additional sources. Principal findings Hereditary hemorrhagic telangiectasia affects 1 in 5000-8000 people. It is a genetic condition in which vascular dysplasia affects many organs particularly the pulmonary, cerebral, gastrointestinal, and spinal vasculature. A large proportion of women with HHT have uneventful pregnancies. However, women can present in pregnancy with clinically silent but potentially life-threatening features of the disorder including fatal hemorrhage from ruptured arteriovenous malformations (AVMs), systemic emboli, and high output cardiac failure secondary to arteriovenous shunting. Literature on the anesthetic management of HHT in pregnancy is limited. Both general and regional anesthetic techniques have been successfully performed in these patients, but are reliant on identifying the presence of specific AVMs; avoidance of cardiovascular instability; and prophylaxis against systemic emboli secondary to pulmonary AVM shunting. The presence of spinal AVMs is considered a relative contraindication to regional techniques. As with other systemic AVMs, these can develop and increase in size during pregnancy with implications for the timing of screening and surveillance. Conclusions An understanding of the presence and potential development of life-threatening AVMs during pregnancy is imperative for anesthesiologists caring for parturients with HHT. Even in the asymptomatic patient, a high index of suspicion should be maintained, screening performed where possible and anesthetic technique adapted accordingly. RésuméObjectif Passer en revue les effets de la te´langiectasie he´morragique he´re´ditaire (THH) chez les parturientes et la prise en charge de telles patientes pendant leur grossesse et l'accouchement. Source Une recherche de la litte´rature

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Life-Threatening Pulmonary Hemorrhage With Pulmonary Arteriovenous Malformations and Hereditary Hemorrhagic Telangiectasia

Cited by 216 publications

References 9 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

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

Anesthetic implications for the parturient with hereditary hemorrhagic telangiectasia

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