title: Impact of pulmonary arteriovenous malformations on respiratory-related quality of life in patients with hereditary haemorrhagic telangiectasia.
abstract: Fifteen to fifty percent of patients with hereditary haemorrhagic telangiectasia have pulmonary arteriovenous malformations. The objective of this study was to measure the effect of the presence of pulmonary arteriovenous malformations and of their embolisation on respiratory-related quality of life (QoL). We prospectively recruited patients with a diagnosis of hereditary haemorrhagic telangiectasia based on the Curaçao criteria and/or the identification of a pathogenic mutation. Respiratory-related quality of life was measured using the Saint George's Respiratory Questionnaire (SGRQ). Patients who underwent embolisation of pulmonary arteriovenous malformations completed the questionnaire before and 6-12 mo after the procedure. The 56 participants were divided into three groups: no pulmonary arteriovenous malformation (group A, n = 10), small pulmonary arteriovenous malformations not accessible to embolotherapy (group B, n = 19), and large pulmonary arteriovenous malformations accessible to embolotherapy (group C, n = 27). The SGRQ score was significantly higher in group C compared to the other groups, indicating a worse respiratory-specific QoL. There was no significant difference between groups A and B. Among the 17 patients who underwent an embolisation, the SGRQ score decreased significantly after the procedure, to a value similar to that in patients without pulmonary arteriovenous malformation. Our results indicate that the presence of large but not small pulmonary arteriovenous malformations negatively affects the respiratory-related quality of life and that embolisation of pulmonary arteriovenous malformations normalizes the respiratory-related quality of life.
authors.affiliation: Aphp, Université De Versailles Saint Quentin En Yvelines, Consultation Pluridisciplinaire Maladie De Rendu-Osler, Hôpital Ambroise Paré, Boulogne, France.
authors.forename: Sandra
authors.initials: S
authors.lastname: Blivet
authors.affiliation: Aphp, Université De Versailles Saint Quentin En Yvelines, Consultation Pluridisciplinaire Maladie De Rendu-Osler, Hôpital Ambroise Paré, Boulogne, France.
authors.forename: Daniel
authors.initials: D
authors.lastname: Cobarzan
authors.affiliation: Aphp, Université De Versailles Saint Quentin En Yvelines, Département De Santé Publique, Hôpital Ambroise Paré, Boulogne, France.
authors.forename: Alain
authors.initials: A
authors.lastname: Beauchet
authors.affiliation: Aphp, Université De Versailles Saint Quentin En Yvelines, Consultation Pluridisciplinaire Maladie De Rendu-Osler, Hôpital Ambroise Paré, Boulogne, France.
authors.forename: Mostafa
authors.initials: M
authors.lastname: El Hajjam
authors.affiliation: Aphp, Université De Versailles Saint Quentin En Yvelines, Consultation Pluridisciplinaire Maladie De Rendu-Osler, Hôpital Ambroise Paré, Boulogne, France.
authors.forename: Pascal
authors.initials: P
authors.lastname: Lacombe
authors.affiliation: Aphp, Université De Versailles Saint Quentin En Yvelines, Consultation Pluridisciplinaire Maladie De Rendu-Osler, Hôpital Ambroise Paré, Boulogne, France.
authors.forename: Thierry
authors.initials: T
authors.lastname: Chinet
mesh_terms: D000328:Adult; D001164:Arteriovenous Fistula / Q000150:complications / Q000503:physiopathology / Q000523:psychology* / Q000601:surgery; D004621:Embolization, Therapeutic; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000503:physiopathology / Q000601:surgery; D011667:Pulmonary Veins / Q000002:abnormalities* / Q000503:physiopathology / Q000601:surgery; D011788:Quality of Life; D012119:Respiration; D012720:Severity of Illness Index; D011795:Surveys and Questionnaires; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications / Q000503:physiopathology / Q000523:psychology* / Q000601:surgery