Author

Tamara Lazic

Date of Award

4-12-2009

Document Type

Open Access Thesis

Degree Name

Medical Doctor (MD)

First Advisor

Bob White

Abstract

Diffuse pulmonary vascular malformations (PAVMs) are a small and understudied, but nevertheless important subset of the PAVM population of patients, associated with significant mortality and morbidity. A review of literature was undertaken to investigate the current understanding of diffuse PAVMs. This review demonstrated that no additional attempts to define diffuse PAVMs and describe their natural history was made before or after the in 2000 report by Faughnan et al {{51 Faughnan,M.E. 2000; }}. To further expand the findings from 2000, we performed a retrospective review of 36 patients (21 female, 15 male) with diffuse PAVMs from a cohort of 821 consecutive patients with PAVMs. Diffuse PAVMs were classified angiographically as involving one or more segmental pulmonary arteries in one or both lungs. The following data were noted from the chart review: Hereditary Hemorrhagic Telangiectasia (HHT) status, gender, age at presentation, presence or absence of large focal PAVMs, oxygen saturations, morbidity and mortality. Twenty nine out of 36 (81%) patients had HHT. Diffuse PAVMs were more commonly bilateral 26/36 (72%) than unilateral 10/36 (28%) (p=0.02). Initial O2 saturations of patients with unilateral and bilateral diffuse PAVM were 87% ± 7% and 79% ± 8% (p=0.02), which the current values are 95% ± 3% and 85% ± 7% (p < .0001) respectively. Nine deaths occurred, but only in patients with bilateral involvement. Deaths were due to hemoptysis from bronchial artery hypertrophy (2), brain hemorrhage (1) and abscess (1), spontaneous liver necrosis (3), operative death during attempted lung transplantation (1), and hemorrhage from duodenal ulcer (1). Yearly follow-up is recommended for this group of patients as they are at high risk for complications.

Comments

This is an Open Access Thesis.

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