Pulmonary arteriovenous malformations in children with hereditary hemorrhagic telangiectasia: a longitudinal study
| Autor: | Lynne A Sekarski, Andrew J. White, Katie L. Mowers, R. Mark Grady |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2018 |
| Předmět: |
pulmonary arteriovenous malformation
Pulmonary and Respiratory Medicine congenital hereditary and neonatal diseases and abnormalities medicine.medical_specialty Longitudinal study lcsh:Diseases of the circulatory (Cardiovascular) system pediatrics medicine.medical_treatment embolization 030204 cardiovascular system & hematology 03 medical and health sciences 0302 clinical medicine hemic and lymphatic diseases pediatric hereditary hemorrhagic telangiectasia otorhinolaryngologic diseases medicine Embolization Pulmonary arteriovenous malformation Telangiectasia lcsh:RC705-779 business.industry lcsh:Diseases of the respiratory system lcsh:RC666-701 Radiology medicine.symptom business 030217 neurology & neurosurgery Research Article |
| Zdroj: | Pulmonary Circulation, Vol 8 (2018) Pulmonary Circulation |
| ISSN: | 2045-8940 |
| Popis: | Pulmonary arteriovenous malformations (PAVMs) often occur in children with hereditary hemorrhagic telangiectasia (HHT). A 14-year longitudinal study of PAVMs in children with HHT was undertaken to assess the prevalence, the clinical impact, and progression of these malformations. This was a retrospective, single-center study from May 2002 to December 2016 of 129 children with HHT diagnosed using Curacao criteria and/or confirmed by genetic testing. Transthoracic contrast echocardiography (TTCE) was the primary screening modality in all patients and PAVMs were diagnosed based on Barzilai criteria. Moderately positive TTCE (Barzilai criteria ≥ 2) was confirmed with subsequent contrast chest CT. New PAVMs were diagnosed with a positive TTCE after an initial negative TTCE. Embolization of PAVMs were performed according to HHT consensus guidelines. Of 129 children with HHT, 76 (59%) were found to have PAVMs. Sixty-seven (88%) were positive for PAVMs on initial screening. Of 63 children without PAVMs on initial screening, 31 were followed for >1 year. Nine of the 31 (29%) developed new PAVMs after initial negative study. Thirty-eight (50%) of the total 76 children with PAVMs had or developed lesions large enough to be treated with embolization. Nine patients with PAVMs initially too small to be treated with embolization, developed progression of disease and ultimately were treated with embolization over time. The majority, 60% (23/38), of the children with large PAVMs had no related clinical symptoms. After embolization, 21% (8/38), of patients underwent repeat interventions. Genetic diagnosis, age, and gender were not associated with risk of having PAVM nor with need for repeat interventions. Nearly 60% of children with HHT develop PAVMs. The risk for new PAVMs to develop, small PAVMs to become large, and previously embolized PAVMs to require further intervention remains throughout childhood. Thus, children with HHT require continued follow-up until adulthood. |
| Databáze: | OpenAIRE |
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