Estimates of maternal risks of pregnancy for women with hereditary haemorrhagic telangiectasia (Osler–Weber–Rendu syndrome): suggested approach for obstetric services

Shovlin, CL; Sodhi, V.; McCarthy, Andrew; Lasjaunias, P.; Jackson, JE; Sheppard, Mary N.

doi:10.1111/j.1471-0528.2008.01786.x

Cited by 161 publications

(231 citation statements)

References 32 publications

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“…While many women with HHT have uneventful pregnancies and straightforward deliveries, the hormonal and cardiovascular changes of pregnancy in these women have been associated with disease progression and presentation of severe complications during this period. 1,3 In one case series of 111 HHT affected women, 13 suffered life-threatening complications of HHT during pregnancy. 3 A number of case reports exist regarding such episodes (Tables 1, 2).…”

Section: Résumémentioning

confidence: 99%

“…1,3 In one case series of 111 HHT affected women, 13 suffered life-threatening complications of HHT during pregnancy. 3 A number of case reports exist regarding such episodes (Tables 1, 2). There is limited literature on HHT in pregnancy and the anesthetic management of such patients during pregnancy and delivery.…”

Section: Résumémentioning

confidence: 99%

“…Such management is usually undertaken at specialist units where screening-surveillance recommendations have been published, particularly when pregnancy is being considered or confirmed. 1,3,8 Many women with HHT are well-informed Tables 1 and 2 of the risks associated in pregnancy from patient group internet sites, including www.telangiectasia.co.uk and www.hht.org. 3 HHT: specific manifestations and pregnancy…”

Section: Hht: Etiology and Diagnosismentioning

confidence: 99%

“…A prior diagnosis of HHT or PAVM was associated with improved survival in the event of a major complication in pregnancy. 3 Indeed, it is recommended that all HHT-affected women should be screened for PAVMs. 3 In many cases, the most useful diagnostic imaging modality is helical computed tomography (CT); more invasive pulmonary angiography is reserved for the treatment of PAVMs.…”

Section: Pulmonary Features Of Hhtmentioning

confidence: 99%

“…3 Indeed, it is recommended that all HHT-affected women should be screened for PAVMs. 3 In many cases, the most useful diagnostic imaging modality is helical computed tomography (CT); more invasive pulmonary angiography is reserved for the treatment of PAVMs. Most of the teratogenic effects of ionizing radiation tend to occur during the first 8-11 weeks of gestation.…”

Section: Pulmonary Features Of Hhtmentioning

confidence: 99%

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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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Section: Résumémentioning

confidence: 99%

Section: Résumémentioning

confidence: 99%

Section: Hht: Etiology and Diagnosismentioning

confidence: 99%

Section: Pulmonary Features Of Hhtmentioning

confidence: 99%

Section: Pulmonary Features Of Hhtmentioning

confidence: 99%

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Anesthetic implications for the parturient with hereditary hemorrhagic telangiectasia

Lomax

Edgcombe

2009

Can J Anesth/J Can Anesth

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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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