Pediatric Pulmonology 46:1121–1127 (2011) Bilateral Lung Transplantation for Pediatric Idiopathic Pulmonary Arterial Hypertension: A Multi-Center Experience G. Schaellibaum, MD, 1 A.E. Lammers, MD, 2 A. Faro, MD, 3 A. Moreno-Galdo, MD, 4 D. Parakininkas, MD, 5 M.G. Schecter, MD, 6 M. Solomon, MD, 7 D. Boyer, MD, 8 C. Conrad, MD, 9 T. Frischer, MD, 10 J. Wong, MD, 11 A. Boehler, MD, 1 and C. Benden, MD 1 * Summary. Many children with idiopathic pulmonary arterial hypertension (IPAH) experience disease progression despite advanced medical therapy. In these children, heart-lung or bilat- eral lung transplantation (BLTx) remain the only therapeutic options when other treatments fail. Data on functional outcome after BLTx in children with IPAH are limited. We report a multi- center experience of BLTx for pediatric IPAH. We performed a retrospective study including 25 centers within the International Pediatric Lung Transplant Collaborative. Children with IPAH who underwent BLTx were included (1996–2006). Twenty-three children underwent BLTx for IPAH, most of whom were in WHO class III or IV level of function pre-transplantation. At 6 months post-transplantation, 82% of children reported improvement in level of function to WHO class I. The median FEV 1 was 89% (12–126) of predicted at 12 months post-transplan- tation. Ten patients (44%) developed BOS at a median of 42 months (3–85), of whom five died at a median of 27 months (4–86) post-transplantation. Overall mortality was 4% at 3 months post-transplantation. The median survival for children in this cohort was 45 months (2–123). Our data suggest that BLTx is a valuable therapeutic option for children with end-stage IPAH with outcomes comparable to that after heart-lung transplantation in children with pulmonary arterial hypertension or those patients undergoing lung transplantation for other indications. In the majority of children, a good cardiopulmonary function is possible following BLTx, making BLTx a good therapeutic option and maximizing donor organ utilization by allowing more hearts to be available for children needing cardiac transplantation. Pediatr Pulmonol. 2011;46:1121–1127. ß 2011 Wiley Periodicals, Inc. Key words: lung transplantation; idiopathic pulmonary arterial hypertension; pediatrics; children. Funding source: none reported. 1 Division of Pulmonary Medicine, University Hospital Zurich, Zurich, Switzerland. 2 Great Ormond Street Hospital for Children, London, United Kingdom. 3 Washington University, St. Louis, Missouri. 4 Hospital Vall D’Hebron, Barcelona, Spain. 5 Children’s Hospital Wisconsin, Milwaukee, Wisconsin. 6 Texas Children’s Hospital, Houston, Texas. 7 Hospital for Sick Children, Toronto, Ontario, Canada. 8 Boston Children’s Hospital, Boston, Massachusetts. 9 Lucile Packard Children’s Hospital at Stanford University, Palo Alto, California. 10 University Children’s Hospital, Vienna, Austria. 11 Stollery Children’s Hospital, Edmonton, Alberta, Canada. Contents of the manuscript were presented at the Annual Meeting of the American Thoracic Society (ATS) 2010 in New Orleans, Louisiana, USA. The authors do not have any competing interest to declare. *Correspondence to: C. Benden, MD, Division of Pulmonary Medicine, University Hospital Zurich, Raemistrasse 100, CH-8091 Zurich, Switzerland. E-mail: christian_benden@yahoo.de Received 30 December 2010; Revised 17 April 2011; Accepted 17 April 2011. DOI 10.1002/ppul.21484 Published online 1 June 2011 in Wiley Online Library (wileyonlinelibrary.com). ß 2011 Wiley Periodicals, Inc.