Follow-up of pulmonary right-to-left shunt in hereditary haemorrhagic telangiectasia.

Autor:	Vorselaars VM; Dept of Cardiology, St Antonius Hospital, Nieuwegein, The Netherlands v.vorselaars@antoniusziekenhuis.nl., Velthuis S; Dept of Cardiology, St Antonius Hospital, Nieuwegein, The Netherlands., Snijder RJ; Dept of Pulmonology, St Antonius Hospital, Nieuwegein, The Netherlands., Westermann CJ; Dept of Pulmonology, St Antonius Hospital, Nieuwegein, The Netherlands., Vos JA; Dept of Interventional Radiology, St Antonius Hospital, Nieuwegein, The Netherlands., Mager JJ; Dept of Pulmonology, St Antonius Hospital, Nieuwegein, The Netherlands., Post MC; Dept of Cardiology, St Antonius Hospital, Nieuwegein, The Netherlands.
Jazyk:	angličtina
Zdroj:	The European respiratory journal [Eur Respir J] 2016 Jun; Vol. 47 (6), pp. 1750-7. Date of Electronic Publication: 2016 Mar 10.
DOI:	10.1183/13993003.01588-2015
Abstrakt:	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. (Copyright ©ERS 2016.)
Databáze:	MEDLINE
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