Incidence of Spontaneous Pulmonary AVM Rupture in HHT Patients

Abstract

The spontaneous rupture of pulmonary AVMs, resulting in pulmonary hemorrhage and hydrothorax, is a life-threatening complication. While this phenomenon has been previously reported, the true incidence is not yet known. This study retrospectively reviewed records of 801 HHT patients with pulmonary AVMs to identify a single lifetime episode of hemothorax or pulmonary hemorrhage secondary to pulmonary AVM rupture. The lifetime prevalence and incidence of pulmonary AVM rupture in HHT patients was 2.7% and 0.16% respectively. In these patients, AVM rupture represented the initial presentation of HHT in nine (40.9%) cases and was life-threatening in nine (40.9%) cases. All cases occurred in virgin lesions, and subsequent embolization was curative. While a feared complication, pulmonary AVM rupture is rare and is likely effectively prevented by existing embolization techniques and indications.

Keywords: hereditary hemorrhagic telangiectasia (HHT); pulmonary AVM; pulmonary AVM rupture.

Figure 1

Flow chart.

Figure 2

Bar graphs demonstrating the locations of other (a) AVMs and (b) co-morbidities in HHT patients with ruptured pulmonary AVMs. (AVM—arteriovenous malformations, CHF—chronic heart failure, COPD—chronic obstructive pulmonary disease, GI—Gastrointestinal).

Figure 3

A 40-year-old female with HHT who presented with hemoptysis. (a) CXR showing large left-sided hemothorax, confirmed on CT, causing collapse of the adjacent lung. (b) Pulmonary angiography demonstrating a complex RLL AVM, followed by coil embolization and resolution of bleeding.