Screening pulmonary arteriovenous malformations in a large cohort of Spanish patients with hemorrhagic hereditary telangiectasia

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• 作者：José ; Antonio Parra^a ; ¹ ; ^{japarra@humv.es" class="auth_mail" title="E-mail the corresponding author} ; José ; Marí ; a Cuesta^b ; ^{jmccbuelna@hotmail.com" class="auth_mail" title="E-mail the corresponding author} ; Roberto Zarrabeitia^c ; ^{roberto.zarrabeitia@scsalud.es" class="auth_mail" title="E-mail the corresponding author} ; Concepció ; n Fariñ ; as-Á ; lvarez^d ; ^{cfarinas@humv.es" class="auth_mail" title="E-mail the corresponding author} ; Jesú ; s Bueno^e ; ^{jesus.bueno@scsalud.es" class="auth_mail" title="E-mail the corresponding author} ; Sarah Marqué ; s^e ; ^{sarah.marques@scsalud.es" class="auth_mail" title="E-mail the corresponding author} ; Carmen Parra-Fariñ ; as^f ; ^{carmenparrafarinas@gmail.com" class="auth_mail" title="E-mail the corresponding author} ; Marí ; a Luisa Botella^g ; ^{cibluisa@cib.csic.es" class="auth_mail" title="E-mail the corresponding author} ; Carmelo Bernabé ; u^g ; ^{bernabeu.c@cib.csic.es" class="auth_mail" title="E-mail the corresponding author} ; Jesú ; s Zarauza^h ; ^{manuel.zarauza@humv.es" class="auth_mail" title="E-mail the corresponding author}
• 关键词：Hereditary hemorrhagic telangiectasia ; Pulmonary arteriovenous malformations ; Transthoracic contrast echocardiography ; Computed tomography ; Ultrasound contrast agents
• 刊名：International Journal of Cardiology
• 出版年：2016
• 出版时间：1 September 2016
• 年：2016
• 卷：218
• 期：Complete
• 页码：240-245
• 全文大小：411 K

文摘

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.

Material and methods

Two hundred and five patients screened for PAVMs using TTCE and computed tomography (CT) performed with an interval of less than 180 days. 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.