Evolving Pulmonary Nodules: Multiple Pulmonary Arteriovenous Fistulas

Hoffman, Richard B.; Rabens, Robert A.

doi:10.2214/ajr.120.4.861

Cited by 18 publications

(4 citation statements)

References 4 publications

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“…12 20-24 Pregnancy has been associated with an increased rate of PAVM growth and its associated complications. [25][26][27][28] An increased growth rate of a PAVM has been attributed to increased blood volume and cardiac output, which leads to increased pulmonary blood flow, preferentially across the low resistance PAVM. 25 The increased blood flow across the PAVM causes its dilatation.…”

Section: Aetiologymentioning

confidence: 99%

Pulmonary arteriovenous malformation

Khurshid

Downie²

2002

Postgraduate Medical Journal

258

298

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Pulmonary arteriovenous malformations (PAVM) are rare pulmonary vascular anomalies. Although most patients are asymptomatic, PAVMs can cause dyspnoea from right-to-left shunt. Because of paradoxical emboli, various central nervous system complications have been described including stroke and brain abscess. There is a strong association between PAVM and hereditary haemorrhagic telangiectasia. Chest radiography and contrast enhanced computed tomography are essential initial diagnostic tools but pulmonary angiography is the gold standard. Contrast echocardiography is useful for diagnosis and monitoring after treatment. Most patients should be treated. Therapeutic options include angiographic embolisation with metal coil or balloon occlusion and surgical excision.

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Section: Aetiologymentioning

confidence: 99%

Pulmonary arteriovenous malformation

Khurshid

Downie²

2002

Postgraduate Medical Journal

258

298

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“…In addition to these series, there have been case reports of HHT complications during pregnancy: PAVM‐related haemorrhage, 14–20 and growth of PAVMs 13,15,16,21,22 have been reported during pregnancy. HHT‐related cerebral AVMs have presented or haemorrhaged in the third trimester 23–25 .…”

Section: Introductionmentioning

confidence: 99%

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

Shovlin¹,

Sodhi²,

McCarthy³

et al. 2008

BJOG

162

204

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Objectives Hereditary haemorrhagic telangiectasia (HHT) affects 1 in 5-8000 individuals. Pregnancy outcomes are rarely reported. The major reason is that most women do not have their HHT diagnosed prior to pregnancy. Using a large well-characterised series, we studied all pregnancies known to have occurred in HHTaffected women, whether or not their diagnosis was known at the time of pregnancy. Our aim was to estimate rates and types of major complications of HHT in pregnancy, to guide management decisions.Design Cohort study, with prospective, retrospective and familial components.Setting/Population Tertiary referral centre population.Methods All 262 pregnancies in the 111 women with HHT and pulmonary arteriovenous malformations (PAVMs) reviewed between 1999 and 2005 were studied. Eighty-two women (74%) did not have a diagnosis of HHT/PAVM at the time of pregnancy. 222 pregnancies in their 86 HHT-affected relatives were also studied.Main outcome measures PAVM bleed, stroke and maternal death.Results Thirteen women experienced life-threatening events during pregnancy: 1.0% (95% CI 0.1-1.9) of pregnancies resulted in a major PAVM bleed; 1.2% (0.3-2.2%) in stroke (not all were HHT related); and 1.0% (0.13-1.9%) in maternal death. All deaths occurred in women previously considered well. In women experiencing a life-threatening event, prior awareness of HHT or PAVM diagnosis was associated with improved survival (P = 0.041, Fisher's exact test).Conclusions Most HHT pregnancies proceed normally. Rare major complications, and improved survival outcome following prior recognition, means that pregnancy in a woman with HHT should be considered high risk. Recommendations for pregnancy management are provided.Keywords Arteriovenous malformations, cerebral, haemorrhage, myocardial infarction, nose bleeds, pulmonary.Please cite this paper as: Shovlin C, Sodhi V, McCarthy A, Lasjaunias P, Jackson J, Sheppard M. Estimates of maternal risks of pregnancy for women with hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu syndrome): suggested approach for obstetric services.

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“…For pregnant PAVM patients, the PAVMs may enlarge 94,96,97,114,115 . At such times, a fall in arterial PaO 2 /SaO 2 may be masked 116 , due to the increase in mixed venous oxygenation resulting from progesterone-stimulated increased minute ventilation (VdotE) 117,118 .…”

Section: Pregnancymentioning

confidence: 99%

“…Occasional patients have presented during pregnancy or post partum with sudden desaturation due to PAVM growth 94,96,97,114,115 . Of particular concern however, is the enhanced risk of PAVM haemorrhage 2,11,94,96,[142][143][144][145] , which may be fatal 11,97 .…”

Section: Pregnancymentioning

confidence: 99%

Pulmonary arteriovenous malformations and other pulmonary aspects of HHT

Shovlin¹,

Wilmshurst²,

Jackson³

2011

Orphan Lung Diseases

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Pulmonary arteriovenous malformations (PAVMs) are vascular structures that provide a direct capillary-free communication between the pulmonary and systemic circulations. The majority of patients have no PAVM-related symptoms, but are at risk of major complications that can be prevented by appropriate interventions. More than 90% of PAVMs occur as part of hereditary haemorrhagic telangiectasia (HHT), the genetic condition most commonly recognised by nosebleeds, anaemia due to chronic haemorrhage, and/or the presence of arteriovenous malformations in pulmonary, hepatic or cerebral circulations. Patients with HHT are also at higher risk of pulmonary hypertension and pulmonary embolic disease, management of which can be compounded by other aspects of their HHT. This chapter primarily addresses PAVMs and pulmonary HHT in the clinical setting, in order to improve patient care. Clinical presentation patterns, diagnostic strategies and management options are presented in detail. Relevant pathophysiological mechanisms discussed include new topics for the PAVM literature, such as the alveolar transit of venous bubbles during diving and contrast echocardiography.

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Evolving Pulmonary Nodules: Multiple Pulmonary Arteriovenous Fistulas

Cited by 18 publications

References 4 publications

Pulmonary arteriovenous malformation

Pulmonary arteriovenous malformation

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

Pulmonary arteriovenous malformations and other pulmonary aspects of HHT

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