Chest
Volume 133, Issue 3, March 2008, Pages 653-661
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ORIGINAL RESEARCH
PULMONARY VASCULAR DISEASE
New Definition and Natural History of Patients With Diffuse Pulmonary Arteriovenous Malformations: Twenty-Seven–Year Experience

https://doi.org/10.1378/chest.07-1949Get rights and content

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.

Section snippets

Demographics and Clinical Presentation

From a cohort of 821 consecutive patients with PAVM seen by the senior author between May 1978 and December 2006, 36 patients had diffuse PAVM and were included in this report. With informed consent and approval of the internal review board, charts were reviewed. HHT status, gender, age at first presentation, the presence of large focal PAVM, degree of diffuse involvement, oxygen saturations, years of follow-up, morbidity, and mortality were noted. The patients were classified into two

Results

Thirty-six of 821 consecutive patients (4.4%) had diffuse PAVM involving one or more segmental pulmonary arteries. Twenty-one patients were female and 15 were male, with a mean age of 35.4 ± 17.8 years (± SD) at the time of presentation. HHT was present in 29 of 36 patients (81%). Mean follow-up was 8.5 ± 6.1 years (range, 0.12 to 26 years). PAVM were morphologically of the type seen in HHT and quite different from the type seen in hepatopulmonary syndrome or abdominal heterotaxy.12

Discussion

Patients with diffuse PAVM represent a small and severely affected subset of patients with PAVM, most of whom have HHT. Very little has been published on these patients since our first attempt to characterize them.7 This project was initiated by our observations that mortality was increasing; and hemoptysis, a relatively infrequent complication in patients with focal PAVM, was frequently seen in patients with diffuse PAVM, irrespective of TCE. These observations are consistent with the

Conclusions

Diffuse PAVM are associated with significant morbidity and mortality. In this article, we define diffuse pulmonary malformation as involvement of a single segmental rather than a whole lobe. Hemoptysis, often occurring with URI, should be treated with antitussives. If hemoptysis progresses, bronchial embolotherapy with large particles should be performed. We recommend yearly follow-up of this group of patients because they are at high risk for complications and they require reassessment on an

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