Pulmonary Arteriovenous Aneurysms and Fistulas: Anatomical Variations, Embryology, and Classification

Anabtawi, Isam N.; Ellison, Robert G.; Ellison, Lois T.

doi:10.1016/s0003-4975(10)66755-0

Cited by 76 publications

(37 citation statements)

References 14 publications

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“…It has been suggested that multiple small PAVM develop as a result of failure of capillary development during fetal development [Anabtawi et al, 1965]. Most (70%) PAVM cases are associated with hereditary hemorrhagic telangiectasia (HHT), an autosomal dominant condition with variable penetrance and expressivity, occurring with a variable incidence (1/30,000 to 1/2,500) according to geographic area [Gossage and Kanj, 1998;Begbie et al, 2003].…”

Section: Discussionmentioning

confidence: 99%

Association of Adams-Oliver syndrome with pulmonary arterio-venous malformation in the same family: A further support to the vascular hypothesis

et al. 2005

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Adams-Oliver syndrome (AOS) is a rare disease characterized by congenital scalp defects, terminal transverse limb defects and cutis marmorata telangiectatica. A significant incidence of cardiac and vascular malformations has been reported, leading to the hypothesis of a vascular defect early involved in the pathogenesis. We report two members of the same family with previously diagnosed AOS based on clinical phenotype and later recognized to have pulmonary arterio-venous malformation (PAVM). None of the subjects fulfilled current diagnostic criteria of hereditary hemorrhagic telangiectasia, which is the most common cause of PAVM. The occurrence of PAVM in AOS lends support to the hypothesis that endothelial specific abnormalities could be a patho-physiological mechanism in its development. Therefore, the role of screening for PAVM in clinical management of subjects with AOS should deserve further studies.

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Section: Discussionmentioning

confidence: 99%

Association of Adams-Oliver syndrome with pulmonary arterio-venous malformation in the same family: A further support to the vascular hypothesis

et al. 2005

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“…Pulmonary arteriovenous fistulas have been postulated to represent the persistence of minute arteriovenous communications present in normal fetal and neonatal lung that enlarge to become fistulous and cause precapillary shunting and arterial desaturation [1]. This theory is based on early histologic studies in which gelatin-calcium carbonate, injected into the pulmonary arteries of stillborn human fetuses, demonstrated vascular channels that bypass the alveolar capillary bed [3].…”

Section: Discussionmentioning

confidence: 99%

Fulminant development of pulmonary arteriovenous fistulas in an infant after total cavopulmonary shunt

et al. 1996

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An infant with cyanotic congenital heart disease and polysplenia syndrome developed profound cyanosis within months of undergoing bilateral cavopulmonary anastomoses. Intrapulmonary shunting was diagnosed by contrast echocardiography with peripheral venous and selective pulmonary artery injection. Histopathology revealed abnormal, thin-walled vessels within the interstitium of the lung lobule. These vessels have not been reported previously and are likely to be the anatomic site of arteriovenous shunting. This case demonstrates that pulmonary arteriovenous fistulas (PAVFs) may develop rapidly after cavopulmonary anastomosis in young infants. It also illustrates the use of contrast echocardiography for following PAVF progression in these patients.

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“…The morphology of PAVMs is variable and could be a large aneurysmal sac, plexiform dilated vascular channels, or a direct communication between artery and vein, which is often dilated and tortuous (24)(25)(26)(27). From the treatment standpoint, White et al (20) classified the PAVMs angioarchitecture as either simple or complex.…”

Section: Discussionmentioning

confidence: 99%

Persistence of pulmonary arteriovenous malformations after successful embolotherapy with Amplatzer vascular plug: long-term results

Aal¹,

Ibrahim²,

Moustafa³

et al. 2016

Diagn Interv Radiol

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Pulmonary Arteriovenous Aneurysms and Fistulas: Anatomical Variations, Embryology, and Classification

Cited by 76 publications

References 14 publications

Association of Adams-Oliver syndrome with pulmonary arterio-venous malformation in the same family: A further support to the vascular hypothesis

Association of Adams-Oliver syndrome with pulmonary arterio-venous malformation in the same family: A further support to the vascular hypothesis

Fulminant development of pulmonary arteriovenous fistulas in an infant after total cavopulmonary shunt

Persistence of pulmonary arteriovenous malformations after successful embolotherapy with Amplatzer vascular plug: long-term results

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