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1University of Toronto, Toronto, Canada, katherine.zukotynski@utoronto.ca 2Department of Medical Imaging, St. Michael's Hospital, University of Toronto, Toronto, Canada, chanr@smh.toronto.on.ca 3Department of Medicine, St. Michael's Hospital, University of Toronto, Toronto, Canada, chowcm@smh.toronto.on.ca 4St. Michaels' Hospital, University of Toronto, Toronto, Canada, cohenj@smh.toronto.on.ca 5Department of Medicine, St. Michael's Hospital, University of Toronto, Toronto, Canada, faughnanm@smh.toronto.on.ca
faughnanm{at}smh.toronto.on.ca
Abstract
BackgroundUntreated pulmonary arteriovenous malformations (PAVMs) can present with life-threatening complications. Agitated saline transthoracic contrast echocardiography (TTCE) has been recommended as the screening test of choice for PAVMs in Hereditary Hemorrhagic Telangiectasia (HHT). A TTCE grading system has been proposed but not validated. The aim of this study was to determine the positive predictive value (PPV) of TTCE grades for the presence of PAVMs on computed tomography (CT).
MethodsA blinded retrospective review was conducted. All patients screened at the Toronto HHT Center (June 2002 - September 2004) with positive TTCE were included. TTCE results were scored for delay (#cardiac cycles) before appearance of microcavitations in the left atrium and graded for intensity of opacification. Grade 1: minimal left ventricular opacification, 2: moderate, 3: extensive without outlining the endocardium, 4: extensive with endocardial definition. Thoracic CT was performed in all and scored as positive, negative or indeterminate for PAVMs.
ResultsOf 155 patients screened for PAVMs, 104 had positive TTCE. Complete data was available in 90 (87%). Mean age was 45 years; 62% were female. Seventeen percent of patients screened and 27% of patients with positive TTCE had CT detectable PAVMs. There was a significant association between TTCE grade and presence of PAVMs on CT (p <0.0001). The PPV of Grades 1, 2, 3 and 4 were 0.02 (95%CI=0.00-0.06), 0.25 (95%CI=0.06-0.44), 0.56 (95%CI=0.23-0.88), and 1.0 (95%CI=1.0-1.0), respectively.
ConclusionsIncreased shunt grade predicts increased probability of PAVM and may be used to guide decisions in the screening algorithm for PAVMs.
Key Words: Arteriovenous malformations Echocardiography Hereditary Hemorrhagic Telangiectasia
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