Follow-up of pulmonary right-to-left shunt in hereditary haemorrhagic telangiectasia. Issue 6 (10th March 2016)

• Record Type: Journal Article
• Authors: Vorselaars, Veronique M.M.; Velthuis, Sebastiaan; Snijder, Repke J.; Westermann, Cornelis J.J.; Vos, Jan A.; Mager, Johannes J.; Post, Martijn C.
• Is Part Of: European respiratory journal. Volume 47:Issue 6(2016)
• Page Start: 1750
• Page End: 1757
• Publication Date: 2016-03-10
• Subjects: Respiratory organs -- Diseases -- Periodicals; Respiration -- Periodicals; 616.2
• DOI: 10.1183/13993003.01588-2015
• Languages: English
• ISSNs: 0903-1936

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. Within 5 years, no treatable PAVMs developed in HHT patients who did not have pulmonary RLS at initial screening http://ow.ly/XoZLQ