Pulmonary Arteriovenous Aneurysm

Lindskog, Gustaf E.; Liebow, Averill A.; Kausel, Harvey W.; Janzen, Arnold H.

doi:10.1097/00000658-195010000-00002

Cited by 63 publications

(13 citation statements)

References 1 publication

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The intensity of shadowing may be diminished or enhanced respectively by the Valsalva and Muller manoeuvres 86. Patients with PAVMs often present with an abnormality on the chest radiograph,55 57 and this may have led to an overestimate of the frequency of radiographic abnormalities at presentation; it is now recognised that a normal posterior-anterior and lateral chest radiograph does not rule out PAVMs (table 3),42 56 87 particularly in patients with small or diffuse malformations.…”

Section: Pulmonary Arteriovenous Malformations and Hhtmentioning

confidence: 99%

“…71The advent of pulmonary artery embolisation110 111altered the risk-benefit ratio of intervention markedly and coincided with a wider recognition of the risks of leaving asymptomatic PAVMs untreated (although such concerns were first raised nearly 50 years ago86). The hazards of intervention relate predominantly to the procedure, though removal of a low resistance shunt may rarely aggravate coincidental pre-existing pulmonary hypertension resulting from nonHHT pulmonary vascular pathogenic events 73…”

Section: Pulmonary Arteriovenous Malformations and Hhtmentioning

confidence: 99%

See 1 more Smart Citation

Rare diseases bullet 4: Hereditary haemorrhagic telangiectasia and pulmonary arteriovenous malformations: issues in clinical management and review of pathogenic mechanisms

Shovlin¹,

Letarte²

1999

Thorax

358

310

View full text Add to dashboard Cite

Hereditary haemorrhagic telangiectasia (HHT, Rendu-Osler-Weber syndrome) exemplifies an important group of diseases which have catalysed advances in the understanding of fundamental pathophysiological mechanisms. In this paper areas of clinical management of HHT are discussed and the molecular pathogenesis is reviewed. The first section is aimed at all clinicians and concentrates on the recognition of a disorder in which silent cerebral and pulmonary involvement may be life threatening if left untreated. Recent data concerning the diagnostic and treatment modalities for pulmonary arteriovenous malformations (PAVMs) are also reviewed, and the growing concern that many patients with HHT may have small or residual PAVMs is highlighted. The paucity of good longitudinal data on these patients and others with diVerent forms of HHT highlights the need for further clinical studies. In the second section the results of molecular research which suggests a role for receptors and ligands of the transforming growth factor (TGF)-superfamily in the pathogenesis of this vascular disease are discussed. The means by which such information may relate to the clinical heterogeneity observed in HHT are specifically addressed, and more fundamental questions such as how reduced cell surface expression of endoglin predisposes a patient to develop PAVMs are also discussed. Hereditary haemorrhagic telangiectasiaThe classical patient with the vascular disorder hereditary haemorrhagic telangiectasia (HHT) has nose bleeds, dilated blood vessels over the lips and finger tips, and gastrointestinal bleeding in later life. However, this clinical scenario represents only one of the presentation patterns of HHT.1 2 It is now recognised that, in addition to microscopic mucocutaneous telangiectases derived from post capillary venules ( fig 1A), 3 HHT leads to the development of larger abnormal vascular structures at other sites. Arteriovenous malformations in the pulmonary, cerebral, and hepatic circulations account for some of the most devastating clinical complications of the disease.The autosomal dominant inheritance pattern of HHT has enabled identification of the underlying genetic defects, prompting increased scientific interest in the disorder. Mutations in at least two genes have been shown to be associated with HHT in diVerent families: endoglin on chromosome 9, 4 and ALK-1 (activin receptor-like kinase 1) on chromosome 12.5 Both genes encode endothelial cell transmembrane proteins that can be defined as components of the receptor complexes for growth factors of the TGF-super- Figure 1 Hereditary haemorrhagic telangiectasia (HHT). (A) Mucocutaneous

show abstract

Section: Pulmonary Arteriovenous Malformations and Hhtmentioning

confidence: 99%

Section: Pulmonary Arteriovenous Malformations and Hhtmentioning

confidence: 99%

Rare diseases bullet 4: Hereditary haemorrhagic telangiectasia and pulmonary arteriovenous malformations: issues in clinical management and review of pathogenic mechanisms

Shovlin¹,

Letarte²

1999

Thorax

358

310

View full text Add to dashboard Cite

show abstract

“…Patients with this illness usually are thought first to be suffering from congenital heart disease, because of their polycythemia, digital clubbing, and cyanosis. 19 Polycythemia vera may also be considered.20 After the heart has been proved normal and after the x-ray has demonstrated a pul¬ monary lesion, the diagnosis of arteriovenous fistula of the lung should be made.…”

Section: Arteriovenus Fistulamentioning

confidence: 99%

Advances in Diagnosis and Treatment of Surgical Pulmonary Disease

Nixon¹

1952

JAMA

View full text Add to dashboard Cite

“…Congenital pulmonary arteriovenous fistula can be effectively treated with either surgical resection [1][2][3] or transcatheter coil embolization [4][5][6][7][8]. In view of the frequent multiplicity and recurrence after treatment, the modest procedure of coil occlusion seems more favorable than surgery [4][5][6][7][8].…”

Section: Introductionmentioning

confidence: 99%