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Reliability of Contrast Echocardiography to Rule Out Pulmonary Arteriovenous Malformations and Avoid CT Irradiation in Pediatric Patients with Hereditary Hemorrhagic Telangiectasia
Author(s) -
Karam Carma,
Sellier Jacques,
Mansencal Nicolas,
Fagnou Carole,
Blivet Sandra,
Chinet Thierry,
Lacombe Pascal,
Dubourg Olivier
Publication year - 2015
Publication title -
echocardiography
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.404
H-Index - 62
eISSN - 1540-8175
pISSN - 0742-2822
DOI - 10.1111/echo.12615
Subject(s) - telangiectasia , medicine , contrast (vision) , radiology , cardiology , surgery , computer science , artificial intelligence
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 ( PAVM s) in children with hereditary hemorrhagic telangiectasia ( HHT ), but the value of TTCE to rule out PAVM s 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 PAVM s 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 PAVM s 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 PAVM s 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 PAVM s 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 PAVM s to be spared the radiation dose of CT .