New definition and natural history of patients with diffuse pulmonary arteriovenous malformations: Twenty-seven-year experience

Paola Pierucci, Joshua Murphy, Katharine J. Henderson, Deborah Chyun, Robert I. White

Research output: Contribution to journalArticle

Abstract

Background: Patients with diffuse pulmonary arteriovenous malformations (PAVM), a small but important subset of the PAVM population, have significant morbidity and mortality rates. Methods: Thirty-six patients (21 female and 15 male) with diffuse PAVM from a cohort of 821 consecutive patients with PAVM were evaluated. Diffuse PAVM were categorized angiographically: involvement of one or more segmental pulmonary arteries in one or both lungs. Hereditary hemorrhagic telangiectasia (HHT) status, gender, presence or absence of large (≥ 3-mm diameter artery) focal PAVM, oxygen saturations, complications including hemoptysis, years of follow-up, and survival were tabulated. Results: HHT was present in 29 of 36 patients (81%), and diffuse PAVM were more commonly bilateral (26 of 36 patients, 72%) than unilateral (10 of 36 patients, 28%) [p = 0.02]. Female gender was associated with bilateral diffuse PAVM (19 of 26 patients, 73%) [p = 0.01]. Focal PAVM were present in both groups but more commonly in patients with bilateral involvement (16 of 26 patients, 62%) [p = 0.02]. Initial oxygen saturations (pulse oximetry, standing) of patients with unilateral and bilateral diffuse PAVM were 87 ± 7% and 79 ± 8% (mean ± SD), respectively (p = 0.02). The last or current values for patients with unilateral and bilateral involvement are 95 ± 3% and 85 ± 7%, respectively (p < 0.0001). Nine deaths occurred, and all were in patients with bilateral involvement. Deaths were due to hemoptysis of bronchial artery origin (n = 2), hemorrhage from duodenal ulcer (n = 1), spontaneous liver necrosis (n = 3), brain hemorrhage (n = 1), brain abscess (n = 1), and operative death during attempted lung transplant (n = 1). Conclusions: Patients with diffuse PAVM are a high-risk group, and yearly follow-up is recommended.

Original languageEnglish (US)
Pages (from-to)653-661
Number of pages9
JournalChest
Volume133
Issue number3
DOIs
StatePublished - Mar 2008

Fingerprint

Arteriovenous Malformations
Natural History
Lung
Hereditary Hemorrhagic Telangiectasia
Hemoptysis
Pulmonary Artery
Bronchial Arteries
Oxygen
Brain Abscess
Oximetry
Intracranial Hemorrhages
Duodenal Ulcer
Necrosis

Keywords

  • Arteriovenous malformations
  • Diffuse
  • Hereditary hemorrhagic telangiectasia
  • Pulmonary angiography
  • Transcatheter embolization

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

New definition and natural history of patients with diffuse pulmonary arteriovenous malformations : Twenty-seven-year experience. / Pierucci, Paola; Murphy, Joshua; Henderson, Katharine J.; Chyun, Deborah; White, Robert I.

In: Chest, Vol. 133, No. 3, 03.2008, p. 653-661.

Research output: Contribution to journalArticle

Pierucci, Paola ; Murphy, Joshua ; Henderson, Katharine J. ; Chyun, Deborah ; White, Robert I. / New definition and natural history of patients with diffuse pulmonary arteriovenous malformations : Twenty-seven-year experience. In: Chest. 2008 ; Vol. 133, No. 3. pp. 653-661.
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title = "New definition and natural history of patients with diffuse pulmonary arteriovenous malformations: Twenty-seven-year experience",
abstract = "Background: Patients with diffuse pulmonary arteriovenous malformations (PAVM), a small but important subset of the PAVM population, have significant morbidity and mortality rates. Methods: Thirty-six patients (21 female and 15 male) with diffuse PAVM from a cohort of 821 consecutive patients with PAVM were evaluated. Diffuse PAVM were categorized angiographically: involvement of one or more segmental pulmonary arteries in one or both lungs. Hereditary hemorrhagic telangiectasia (HHT) status, gender, presence or absence of large (≥ 3-mm diameter artery) focal PAVM, oxygen saturations, complications including hemoptysis, years of follow-up, and survival were tabulated. Results: HHT was present in 29 of 36 patients (81{\%}), and diffuse PAVM were more commonly bilateral (26 of 36 patients, 72{\%}) than unilateral (10 of 36 patients, 28{\%}) [p = 0.02]. Female gender was associated with bilateral diffuse PAVM (19 of 26 patients, 73{\%}) [p = 0.01]. Focal PAVM were present in both groups but more commonly in patients with bilateral involvement (16 of 26 patients, 62{\%}) [p = 0.02]. Initial oxygen saturations (pulse oximetry, standing) of patients with unilateral and bilateral diffuse PAVM were 87 ± 7{\%} and 79 ± 8{\%} (mean ± SD), respectively (p = 0.02). The last or current values for patients with unilateral and bilateral involvement are 95 ± 3{\%} and 85 ± 7{\%}, respectively (p < 0.0001). Nine deaths occurred, and all were in patients with bilateral involvement. Deaths were due to hemoptysis of bronchial artery origin (n = 2), hemorrhage from duodenal ulcer (n = 1), spontaneous liver necrosis (n = 3), brain hemorrhage (n = 1), brain abscess (n = 1), and operative death during attempted lung transplant (n = 1). Conclusions: Patients with diffuse PAVM are a high-risk group, and yearly follow-up is recommended.",
keywords = "Arteriovenous malformations, Diffuse, Hereditary hemorrhagic telangiectasia, Pulmonary angiography, Transcatheter embolization",
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T1 - New definition and natural history of patients with diffuse pulmonary arteriovenous malformations

T2 - Twenty-seven-year experience

AU - Pierucci, Paola

AU - Murphy, Joshua

AU - Henderson, Katharine J.

AU - Chyun, Deborah

AU - White, Robert I.

PY - 2008/3

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N2 - Background: Patients with diffuse pulmonary arteriovenous malformations (PAVM), a small but important subset of the PAVM population, have significant morbidity and mortality rates. Methods: Thirty-six patients (21 female and 15 male) with diffuse PAVM from a cohort of 821 consecutive patients with PAVM were evaluated. Diffuse PAVM were categorized angiographically: involvement of one or more segmental pulmonary arteries in one or both lungs. Hereditary hemorrhagic telangiectasia (HHT) status, gender, presence or absence of large (≥ 3-mm diameter artery) focal PAVM, oxygen saturations, complications including hemoptysis, years of follow-up, and survival were tabulated. Results: HHT was present in 29 of 36 patients (81%), and diffuse PAVM were more commonly bilateral (26 of 36 patients, 72%) than unilateral (10 of 36 patients, 28%) [p = 0.02]. Female gender was associated with bilateral diffuse PAVM (19 of 26 patients, 73%) [p = 0.01]. Focal PAVM were present in both groups but more commonly in patients with bilateral involvement (16 of 26 patients, 62%) [p = 0.02]. Initial oxygen saturations (pulse oximetry, standing) of patients with unilateral and bilateral diffuse PAVM were 87 ± 7% and 79 ± 8% (mean ± SD), respectively (p = 0.02). The last or current values for patients with unilateral and bilateral involvement are 95 ± 3% and 85 ± 7%, respectively (p < 0.0001). Nine deaths occurred, and all were in patients with bilateral involvement. Deaths were due to hemoptysis of bronchial artery origin (n = 2), hemorrhage from duodenal ulcer (n = 1), spontaneous liver necrosis (n = 3), brain hemorrhage (n = 1), brain abscess (n = 1), and operative death during attempted lung transplant (n = 1). Conclusions: Patients with diffuse PAVM are a high-risk group, and yearly follow-up is recommended.

AB - Background: Patients with diffuse pulmonary arteriovenous malformations (PAVM), a small but important subset of the PAVM population, have significant morbidity and mortality rates. Methods: Thirty-six patients (21 female and 15 male) with diffuse PAVM from a cohort of 821 consecutive patients with PAVM were evaluated. Diffuse PAVM were categorized angiographically: involvement of one or more segmental pulmonary arteries in one or both lungs. Hereditary hemorrhagic telangiectasia (HHT) status, gender, presence or absence of large (≥ 3-mm diameter artery) focal PAVM, oxygen saturations, complications including hemoptysis, years of follow-up, and survival were tabulated. Results: HHT was present in 29 of 36 patients (81%), and diffuse PAVM were more commonly bilateral (26 of 36 patients, 72%) than unilateral (10 of 36 patients, 28%) [p = 0.02]. Female gender was associated with bilateral diffuse PAVM (19 of 26 patients, 73%) [p = 0.01]. Focal PAVM were present in both groups but more commonly in patients with bilateral involvement (16 of 26 patients, 62%) [p = 0.02]. Initial oxygen saturations (pulse oximetry, standing) of patients with unilateral and bilateral diffuse PAVM were 87 ± 7% and 79 ± 8% (mean ± SD), respectively (p = 0.02). The last or current values for patients with unilateral and bilateral involvement are 95 ± 3% and 85 ± 7%, respectively (p < 0.0001). Nine deaths occurred, and all were in patients with bilateral involvement. Deaths were due to hemoptysis of bronchial artery origin (n = 2), hemorrhage from duodenal ulcer (n = 1), spontaneous liver necrosis (n = 3), brain hemorrhage (n = 1), brain abscess (n = 1), and operative death during attempted lung transplant (n = 1). Conclusions: Patients with diffuse PAVM are a high-risk group, and yearly follow-up is recommended.

KW - Arteriovenous malformations

KW - Diffuse

KW - Hereditary hemorrhagic telangiectasia

KW - Pulmonary angiography

KW - Transcatheter embolization

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