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European Journal of Echocardiography Advance Access published online on December 17, 2008
European Journal of Echocardiography, doi:10.1093/ejechocard/jen317
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Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2008. For permissions please email: journals.permissions@oxfordjournals.org

Contrast echocardiography for pulmonary arteriovenous malformations screening: does any bubble matter?

P. Gazzaniga1, E. Buscarini2,*, G. Leandro3, L. Reduzzi4, M. Grosso5, G. Pongiglione6, C. Pedrinazzi1, L. Lanzarini7, V. Portugalli4, P. Blotta8, P. Forner8, E. Boccardi9, F. Pagella10, G. Manfredi2, C. Olivieri11, A. Zambelli2, C. Danesino11 and G. Inama1

1 Cardiology Department, Maggiore Hospital, Crema, Italy
2 Gastroenterology Department, Maggiore Hospital, Largo Dossena 2, 26013 Crema, Italy
3 Gastroenterology Dept 1, IRCCS, Castellana Grotte, Italy
4 Radiology Department, Maggiore Hospital, Crema, Italy
5 Radiology Department, Santa Croce Hospital, Cuneo, Italy
6 Pediatric Cardiology Department, Hospital Gaslini, Genova, Italy
7 Cardiology Department, Policlinico S Matteo, Pavia, Italy
8 ENT Department, Maggiore Hospital, Crema, Italy
9 Interventional Neuroradiology, Niguarda Hospital, Milan, Italy
10 ENT Department, Policlinico S Matteo, Pavia, Italy
11 Genetic Institute, University of Pavia, Pavia, Italy

Received 19 July 2008; accepted after revision 22 November 2008.

* Corresponding author. Tel: +39 0373 280278; fax: +39 0373 280654. E-mail address: ebuscarini{at}rim.it


  Abstract

Aims: To evaluate diagnostic accuracy of contrast echocardiography (CE) as compared with CT, for the screening of pulmonary arteriovenous malformations (PAVMs) in hereditary haemorrhagic telangiectasia (HHT); to evaluate the clinical significance of semi-quantitative analysis of a shunt on CE.

Methods and results: A blinded prospective study was conducted in 190 consecutive subjects at risk of HHT who underwent screening for PAVMs, including clinical evaluation, pulse oximetry, standard and CE, and chest multirow CT without contrast medium. A semi-quantitative analysis of the shunt size was performed according to the contrast echo opacification of the left-sided chambers: Grade 0, no bubbles; 1, occasional filling with <20 bubbles; 2, moderate filling; 3, complete opacification. The first 100 patients were compared with 100 controls. A total of 119 (63%) patients had positive CE (32.2% Grade 1, 13.1% Grade 2, 11% Grade 3, 6.3% with patent foramen ovale). The overall diagnostic performance of CE was sensitivity 1.00, specificity 0.49, positive predictive value (PPV) 0.32, negative predictive value (NPV) 1.00. The PPV for the different grades was 0.00 for Grade 1, 0.56 for Grade 2, 1.00 for Grade 3; the NPV of Grade 0 was 1.00. A significant correlation was found between the CE grading and the number of PAVM, and complications (P < 0.0001).

Conclusion: CE is an extremely sensitive procedure for the detection of PAVMs with substantial clinical impact.

Keywords: Arteriovenous malformations; Echocardiography; Hereditary haemorrhagic telangiectasia


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