PULMONARY ARTERIOVENOUS MALFORMATIONS IN CHILDREN: OUTCOMES OF TRANSCATHETER EMBOLOTHERAPY MARIE E. FAUGHNAN, MD, ASHRAF THABET, BA, MEIR MEI-ZAHAV, MD, MARIA COLOMBO, MD, I AN MACLUSKY, MD, ROBERT H. HYLAND, MD, ROBYN A. PUGASH, MD, PETER CHAIT, MD, KATHARINE J. HENDERSON, MS, AND ROBERT I. WHITE,J R, MD Objective To describe outcomes of transcatheter embolotherapy (TCE) in children with pulmonary arteriovenous malformations (PAVMs). Study design Chart and imaging review of all children (age #18 years) treated for PAVMs by TCE at three hereditary hemorrhagic telangiectasia centers. Results All 42 treated patients were included, with a mean age of 12 years (range, 4 to 18). Cyanosis was present in 25 of 42 patients (60%). Hemoptysis had occurred in 3 of 42 patients (7%) and neurologic complications (stroke, cerebral abscess) occurred in 8 patients (19%) before assessment. PAVMs were focal in 30 of 42 (71%) and diffuse in 12 of 42 (29%) patients. TCE was performed for 172 PAVMs and 35 diffuse regions (regional TCE). Follow-up was obtained in 38 of 42 (90%) patients (mean, 7 years). After TCE in patients with focal PAVMs, oxygenation improved significantly, with no further complications from the PAVMs. Reperfusion was noted in 23 of 153 (15%) PAVMs. Eighteen of 23 (78 %) of these were retreated, with documented aneurysmal involution in 10 of 13 (77%) patients. TCE complications included pleuritic chest pain (24% of sessions) and deployment complications (device paradoxical embolization or device misplacement) (3% of sessions, 1% of PAVMs), with no long-term complications. Conclusions PAVMs cause life-threatening complications in children; treatment with TCE is safe, with complication rates comparable to adult rates. (J Pediatr 2004;145:826-31) P ulmonary arteriovenous malformations (PAVMs), are present in at least 30% of adults with hereditary hemorrhagic telangiectasia (HHT), 1 and they have been well characterized. 2-5 Patients with PAVMs are frequently asymptomatic; therefore PAVMs often remain unrecognized until a serious complication develops, such as stroke or cerebral abscess. Neurologic complications occur in approximately 40% of adults with untreated PAVMs and hemorrhagic complications (massive hemoptysis or spontaneous hemothorax) occur in approximately 15%. 2-5 Fortunately, sensitive screening methods have been described, 1 and therapy exists that is both safe and effective. The treatment of choice for PAVMs in adults is transcatheter embolotherapy (TCE). 2,4,6,7 Only case reports and small case series of up to 5 patients describe the clinical presentation of PAVMs in children, and few report any treatment or outcomes. To date, only 6 case reports of TCE in 8 children 4,8-12 have been published. Five of the 8 reported good results; no follow-up was reported for the other 3 cases. HHT is increasingly recognized in adults in whom screening and treatment for PAVMs has become the standard of care. Genetic diagnosis has recently become available; therefore we expect increasing numbers of asymptomatic children to be diagnosed with HHT, many of whom will be diagnosed with asymptomatic PAVMs. The clinical presentation of PAVMs and the safety and effectiveness of TCE must therefore be evaluated specifically in children to clarify the role of screening and treatment for PAVMs in children of HHT families. From the Department of Medicine, Division of Respiratory Medicine, St Michael’s Hospital, University of Toronto, Toronto, Ontario, Canada; the Department of Di- agnostic Radiology, Yale University School of Medicine, New Haven, Connecticut; the Department of Pediatrics, Division of Re- spiratory Medicine, and the Department of Medical Imaging, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada; the Department of Diagnostic Radiology, Good Samaritan Regional Med- ical Center, Phoenix, Arizona; and the Department of Medical Imaging, Sunny- brook and Women’s College Health Scien- ces Centre, University of Toronto, Toronto, Ontario, Canada. Supported by the Squires Club, Nelson Arthur Hyland Foundation, Sonor Founda- tion, St Michael’s Hospital Research In- stitute (M.E.F.), and March of Dimes grant HHT-FY03-677, Josephine Lawrence Hop- kins Foundation, and General Clinical Re- search Center NIH grant M01-RR-00125 (R.I.W., K.H., and A.T.). Submitted for publication Jul 7, 2003; last revision received Apr 19, 2004; accepted Aug 9, 2004. Reprint requests: Dr Marie E. Faughnan, St Michael’s Hospital, 30 Bond St, Suite 6045, Toronto, Ontario, Canada M5B-1W8. E-mail: faughnanm@smh.toronto.on.ca. 0022-3476/$ - see front matter Copyright ª 2004 Elsevier Inc. All rights reserved. 10.1016/j.jpeds.2004.08.046 CP Collateral perfusion HHT Hereditary hemorrhagic telangiectasia PAVMs Pulmonary arteriovenous malformations TCE Transcatheter embolotherapy SpO 2 Oxygen saturation by pulse oximetry 826