title: Screening pulmonary arteriovenous malformations in a large cohort of Spanish patients with hemorrhagic hereditary telangiectasia.
abstract: OBJECTIVE
  Because of the serious nature of potential complications, screening for pulmonary arteriovenous malformations is required in patients with hereditary hemorrhagic telangiectasia. The aim of this study was to evaluate the utility of contrast echocardiography and compare the performance of two contrast agents: agitated saline and Gelofusine.
  
  
  METHODS
  Two hundred and five patients screened for PAVMs using TTCE and computed tomography (CT) performed with an interval of less than 180days. Contrast echocardiography studies were graded on a 4-point semiquantitative scale based on the amount of microbubbles seen in left heart chambers.
  
  
  RESULTS
  Positive TTCE findings were seen in 137 (66.8%) patients, whereas CT confirmed PAVMs in 59 (43.1%). Two of 67 grade 1 patients; 18 of 42 grade 2; 17 of 22 grade 3 and all grade 4 had PAVMs on CT. Embolotherapy was feasible in 38.9% patients in grade 2 and 82.3% and 95.2% in grades 3-4. No patients in grade 1 were embolized. The mean cardiac cycle in which bubbles were first seen in the left heart in patients without and with PAVMs on CT was 6.1 and 3.9 (p<0.0001). Compared to saline, Gelofusine produced an overall increase in grade.
  
  
  CONCLUSIONS
  No grade 1 patients had treatable PAVMs. There is a need for improvement in the selection of patients for CT in grade 2, where less than half have PAVMs on CT. The cardiac cycle may help to differentiate between patients with and without PAVMs. Gelofusine was not better than saline for PAVM screening.
authors.affiliation: Department of Radiology Hospital Universitario Marqués de Valdecilla, Santander, Cantabria, Spain. Electronic address: japarra@humv.es.
authors.forename: José Antonio
authors.initials: JA
authors.lastname: Parra
authors.affiliation: Department of Cardiology, Hospital Sierrallana, Torrelavega, Cantabria, Spain. Electronic address: jmccbuelna@hotmail.com.
authors.forename: José María
authors.initials: JM
authors.lastname: Cuesta
authors.affiliation: Department of Internal Medicine, Hospital Sierrallana and Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Torrelavega, Cantabria, Spain. Electronic address: roberto.zarrabeitia@scsalud.es.
authors.forename: Roberto
authors.initials: R
authors.lastname: Zarrabeitia
authors.affiliation: Quality Unit, Hospital Universitario Marques de Valdecilla, Santander, Cantabria, Spain. Electronic address: cfarinas@humv.es.
authors.forename: Concepción
authors.initials: C
authors.lastname: Fariñas-Álvarez
authors.affiliation: Department of Radiology, Hospital Sierrallana, Torrelavega, Cantabria, Spain. Electronic address: jesus.bueno@scsalud.es.
authors.forename: Jesús
authors.initials: J
authors.lastname: Bueno
authors.affiliation: Department of Radiology, Hospital Sierrallana, Torrelavega, Cantabria, Spain. Electronic address: sarah.marques@scsalud.es.
authors.forename: Sarah
authors.initials: S
authors.lastname: Marqués
authors.affiliation: Department of Radiology, Hospital Universitari Vall d´Hebron, Barcelona, Catalonia, Spain. Electronic address: carmenparrafarinas@gmail.com.
authors.forename: Carmen
authors.initials: C
authors.lastname: Parra-Fariñas
authors.affiliation: Centro de Investigaciones Biológicas, Consejo Superior de Investigaciones Científicas (CSIC), and Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Madrid, Spain. Electronic address: cibluisa@cib.csic.es.
authors.forename: María Luisa
authors.initials: ML
authors.lastname: Botella
authors.affiliation: Centro de Investigaciones Biológicas, Consejo Superior de Investigaciones Científicas (CSIC), and Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Madrid, Spain. Electronic address: bernabeu.c@cib.csic.es.
authors.forename: Carmelo
authors.initials: C
authors.lastname: Bernabéu
authors.affiliation: Department of Cardiology, Hospital Universitario Marqués de Valdecilla, Santander, Cantabria, Spain. Electronic address: manuel.zarauza@humv.es.
authors.forename: Jesús
authors.initials: J
authors.lastname: Zarauza
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001164:Arteriovenous Fistula / Q000000981:diagnostic imaging*; D001165:Arteriovenous Malformations / Q000000981:diagnostic imaging*; D002648:Child; D002675:Child, Preschool; D003287:Contrast Media; D004452:Echocardiography / Q000379:methods*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000000981:diagnostic imaging; D011667:Pulmonary Veins / Q000002:abnormalities* / Q000000981:diagnostic imaging; D013030:Spain; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications*; D014057:Tomography, X-Ray Computed / Q000379:methods*; D055815:Young Adult
chemical_list: D003287:Contrast Media
keywords: Computed tomography; Hereditary hemorrhagic telangiectasia; Pulmonary arteriovenous malformations; Transthoracic contrast echocardiography; Ultrasound contrast agents