RT Journal Article
SR Electronic
T1 Follow-up of the pulmonary right-to-left shunt with transthoracic contrast echocardiography in patients with hereditary haemorrhagic telangiectasia
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP PA2106
DO 10.1183/13993003.congress-2015.PA2106
VO 46
IS suppl 59
A1 Veronique M.M. Vorselaars
A1 Sebas Velthuis
A1 Repke J. Snijder
A1 Cornelis J.J. Westermann
A1 Jan-Albert Vos
A1 Johannes J. Mager
A1 Marco C. Post
YR 2015
UL http://erj.ersjournals.com/content/46/suppl_59/PA2106.abstract
AB Introduction: Pulmonary arteriovenous malformations (PAVMs) are associated with severe neurological complications in patients with hereditary haemorrhagic telangiectasia (HHT). Transthoracic contrast echocardiography (TTCE) is the first-line screening technique for the detection of pulmonary right-to-left shunts (RLS) and only moderate and large shunts seem to have clinical implications.Objectives: Five years after the initial TTCE we evaluated the evolution of the pulmonary RLS in a single centre cohort.Methods: All HHT patients underwent a second TTCE five years after screening. Patients with a history of PAVM embolisation were excluded. Opacification of the left ventricle was graded with a three grade scale. The TTCE after 5 years was compared to the TTCE performed at screening.Results: In total 162 patients (55% female, 65.4% HHT type 2, age at follow-up 50.6±14.0 years) were included (expected patient number at presentation 190). The median follow-up time was 5.4 years (interquartile range 5.1-5.9 years). A pulmonary RLS was present in 93 patients (57.4%) at screening and 104 patients (64.2%) at follow-up. Increase in shunt grade was seen in 27 patients (16.7%). A significant increase was seen in 15 of these patients (55.6%) and embolisation was indicated in 6 patients (22.2%). Embolisation was feasible in 3 patients (1.9%) in whom the shunt increased within one grade but with increase of the PAVM on computed tomography. There were no complications.Conclusions: Even in patients with no treatable PAVMs at screening, after 5 years treatable PAVMs are present in 6%. In this population there is a number-needed-to-screen of 18.