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Pulmonary Arteriovenous Malformations^*

Screening Procedures and Pulmonary Angiography in Patients With Hereditary Hemorrhagic Telangiectasia

^* From the Department of Otorhinolaryngology (Drs. Kjeldsen and Vase), Svendborg Hospital, Svendborg, Denmark; and the Paediatric Cardiology Unit (Dr. Oxhøj), the Department of Radiology (Drs. Andersen and Elle), and the Department of Otorhinolaryngology (Drs. Kjeldsen and Jacobsen), Odense University Hospital, Odense, Denmark.

Correspondence to: Anette Drøhse Kjeldsen, MD, PhD, Department of Otorhinolaryngology, Odense University Hospital, DK-5000 Odense C, Denmark; e-mail: ad.kjeldsen{at}ishmed.ou.dk

Background: Hereditary hemorrhagic telangiectasia (HHT) is a dominantly inherited disease with a high prevalence of pulmonary arteriovenous malformations (PAVMs). The first symptom of HHT may be stroke or fatal hemoptysis associated with the presence of PAVM.

Objective: To evaluate different screening methods applied for the identification of PAVMs.

Setting: Odense University Hospital.

Subjects: HHT patients with positive findings on contrast echocardiography (CE) who participated in a screening investigation and underwent pulmonary angiography (PA).

Methods: Different screening methods were evaluated against the results of PA. In a group of patients with positive findings on CE, we compared results of PA with the following: severity of dyspnea; results of pulse oximetry arterial oxygen saturation (SaO₂) supine and upright; supine PaO₂ in room air and while breathing 100% oxygen; size of arteriovenous shunt in supine position; chest radiograph; and intensity of contrast at CE.

Results: PA was performed in 25 HHT patients with positive findings on CE, 15 of whom had PAVM. Embolization therapy was recommended in 12 patients, and 3 patients had small PAVMs not accessible for therapy. In 10 patients, PAVM could not be demonstrated at PA. The sensitivity and specificity calculated for the screening procedures are as follows: 53% and 90%, respectively, for SaO₂; 60% and 100%, respectively, for chest radiograph; 73% and 80%, respectively, for PaO₂ in room air; 100% and 40%, respectively, for PaO₂ breathing 100% oxygen; and 64% and 80%, respectively, for shunt measurement.

Conclusion: Initial screening with CE followed by measurement of PaO₂ while breathing 100% oxygen seemed to be the best screening procedure for identification of patients with PAVM. Screening with chest radiograph and pulse oximetry was shown to be insufficient.

Key Words: hereditary hemorrhagic telangiectasia • pulmonary arteriovenous malformation • pulmonary shunt • Rendu-Osler-Weber syndrome • screening

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