Chest
ORIGINAL RESEARCHPULMONARY VASCULAR DISEASENew Definition and Natural History of Patients With Diffuse Pulmonary Arteriovenous Malformations: Twenty-Seven–Year Experience
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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