title: Reliability of contrast echocardiography to rule out pulmonary arteriovenous malformations and avoid CT irradiation in pediatric patients with hereditary hemorrhagic telangiectasia.
abstract: BACKGROUND
  The overall risk of cancer is higher in people exposed to computed tomography (CT) scans in childhood or adolescence compared to adults. Transthoracic contrast echocardiography (TTCE) has recently been used to screen for pulmonary arteriovenous malformations (PAVMs) in children with hereditary hemorrhagic telangiectasia (HHT), but the value of TTCE to rule out PAVMs and avoid chest CT radiation has yet to be discussed.
  
  
  METHODS
  Between 2003 and 2013, 92 pediatric patients with ≥3 Curaçao criteria and/or genetic mutation underwent TTCE and chest CT on the same day. We used the classification proposed by Barzilai for TTCE quantification of shunting. We considered CT findings as negative when no PAVMs or only one microscopic PAVM was detected.
  
  
  RESULTS
  Mean age was 11.2 ± 4.1 years. The shunt was grade 0 on TTCE in 27.3%, grade 1 in 17%, grade 2 in 29.6%, grade 3 in 23.9%, and grade 4 in 2.2%. We found PAVMs on chest CT in 52.2%. All the patients with a grade 0 or 1 had a negative CT. The sensitivity and specificity of TTCE for the detection of PAVMs were 100% and 95.1%, respectively. The negative predictive value (NPV) was 100% and the positive predictive value (PPV) was 96%.
  
  
  CONCLUSIONS
  A low-grade classification (Barzilai 0 or 1) could presumably exclude the presence of PAVMs and allow CT irradiation to be avoided in children and adolescents. The screening algorithm using TTCE first would allow more than 40% of the pediatric patients screened for PAVMs to be spared the radiation dose of CT.
authors.affiliation: Cardiology Department, Reference Center for Hereditary Heart Disease, Hospital Ambroise Paré AP-HP Boulogne-Billancourt, University of Versailles, Saint Quentin en Yvelines, France.
authors.forename: Carma
authors.initials: C
authors.lastname: Karam
authors.forename: Jacques
authors.initials: J
authors.lastname: Sellier
authors.forename: Nicolas
authors.initials: N
authors.lastname: Mansencal
authors.forename: Carole
authors.initials: C
authors.lastname: Fagnou
authors.forename: Sandra
authors.initials: S
authors.lastname: Blivet
authors.forename: Thierry
authors.initials: T
authors.lastname: Chinet
authors.forename: Pascal
authors.initials: P
authors.lastname: Lacombe
authors.forename: Olivier
authors.initials: O
authors.lastname: Dubourg
mesh_terms: D001164:Arteriovenous Fistula / Q000150:complications / Q000000981:diagnostic imaging*; D002648:Child; D002675:Child, Preschool; D003287:Contrast Media; D004452:Echocardiography / Q000379:methods*; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D011651:Pulmonary Artery / Q000002:abnormalities* / Q000000981:diagnostic imaging; D011667:Pulmonary Veins / Q000002:abnormalities* / Q000000981:diagnostic imaging; D011835:Radiation Protection / Q000379:methods*; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity; D013683:Telangiectasia, Hereditary Hemorrhagic / Q000150:complications / Q000000981:diagnostic imaging*; D014057:Tomography, X-Ray Computed
chemical_list: D003287:Contrast Media
keywords: cardiac imaging; contrast echocardiography; contrast microbubbles; hereditary hemorrhagic telangiectasia; pediatrics; pulmonary arteriovenous malformation; right-to-left shunt