Bilateral lung transplantation for pediatric idiopathic pulmonary arterial hypertension: a multi-center experience. Pediatr Pulmonol 2011 Nov;46(11):1121-7
Date
06/03/2011Pubmed ID
21634032DOI
10.1002/ppul.21484Scopus ID
2-s2.0-80055022640 (requires institutional sign-in at Scopus site) 14 CitationsAbstract
Many children with idiopathic pulmonary arterial hypertension (IPAH) experience disease progression despite advanced medical therapy. In these children, heart-lung or bilateral 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-transplantation. 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.
Author List
Schaellibaum G, Lammers AE, Faro A, Moreno-Galdo A, Parakininkas D, Schecter MG, Solomon M, Boyer D, Conrad C, Frischer T, Wong J, Boehler A, Benden CAuthor
Daiva E. Parakininkas MD Associate Professor in the Pediatrics department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
AdolescentChild
Child, Preschool
Familial Primary Pulmonary Hypertension
Female
Humans
Hypertension, Pulmonary
Infant
Lung
Lung Transplantation
Male
Retrospective Studies
Treatment Outcome