title: Follow-up of pulmonary right-to-left shunt in hereditary haemorrhagic telangiectasia.
abstract: Pulmonary arteriovenous malformations (PAVMs) are associated with severe neurological complications in hereditary haemorrhagic telangiectasia (HHT). Transthoracic contrast echocardiography (TTCE) is recommended for screening of pulmonary right-to-left shunts (RLS). Although growth of PAVMs is shown in two small studies, no studies on follow-up with TTCE exist.All HHT patients underwent a second TTCE 5 years after initial screening. Patients with a history of PAVM embolisation were excluded. Pulmonary RLS grade on TTCE after 5 years was compared to the grade at screening.200 patients (53.5% female, mean±sd age at screening 44.7±14.1 years) were included. Increase in RLS grade occurred in 36 (18%) patients, of whom six (17%) underwent embolisation. The change in grade between screening and follow-up was not more than one grade. Of patients with nontreatable pulmonary RLS at screening (n=113), 14 (12.4%) underwent embolisation. In patients without pulmonary RLS at initial screening (n=87), no treatable PAVMs developed during follow-up.Within 5 years, no treatable PAVMs developed in HHT patients without pulmonary RLS at initial screening. Increase in pulmonary RLS grade occurred in 18% of patients, and never increased by more than one grade. Of patients with nontreatable pulmonary RLS at initial screening, 12% underwent embolisation.
authors.affiliation: Dept of Cardiology, St Antonius Hospital, Nieuwegein, The Netherlands v.vorselaars@antoniusziekenhuis.nl.
authors.forename: Veronique M M
authors.initials: VM
authors.lastname: Vorselaars
authors.affiliation: Dept of Cardiology, St Antonius Hospital, Nieuwegein, The Netherlands.
authors.forename: Sebastiaan
authors.initials: S
authors.lastname: Velthuis
authors.affiliation: Dept of Pulmonology, St Antonius Hospital, Nieuwegein, The Netherlands.
authors.forename: Repke J
authors.initials: RJ
authors.lastname: Snijder
authors.affiliation: Dept of Pulmonology, St Antonius Hospital, Nieuwegein, The Netherlands.
authors.forename: Cornelis J J
authors.initials: CJ
authors.lastname: Westermann
authors.affiliation: Dept of Interventional Radiology, St Antonius Hospital, Nieuwegein, The Netherlands.
authors.forename: Jan A
authors.initials: JA
authors.lastname: Vos
authors.affiliation: Dept of Pulmonology, St Antonius Hospital, Nieuwegein, The Netherlands.
authors.forename: Johannes J
authors.initials: JJ
authors.lastname: Mager
authors.affiliation: Dept of Cardiology, St Antonius Hospital, Nieuwegein, The Netherlands.
authors.forename: Martijn C
authors.initials: MC
authors.lastname: Post
mesh_terms: D000328:Adult; D001164:Arteriovenous Fistula / Q000000981:diagnostic imaging* / Q000503:physiopathology*; D001165:Arteriovenous Malformations; D003287:Contrast Media / Q000737:chemistry; D004452:Echocardiography; D004621:Embolization, Therapeutic; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008168:Lung / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000503:physiopathology; D011667:Pulmonary Veins / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000503:physiopathology; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications / Q000000981:diagnostic imaging* / Q000503:physiopathology*; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D003287:Contrast Media