title: Bilateral lung transplantation for pediatric idiopathic pulmonary arterial hypertension: a multi-center experience.
abstract: 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.
authors.affiliation: Division of Pulmonary Medicine, University Hospital Zurich, Zurich, Switzerland.
authors.forename: G
authors.initials: G
authors.lastname: Schaellibaum
authors.forename: A E
authors.initials: AE
authors.lastname: Lammers
authors.forename: A
authors.initials: A
authors.lastname: Faro
authors.forename: A
authors.initials: A
authors.lastname: Moreno-Galdo
authors.forename: D
authors.initials: D
authors.lastname: Parakininkas
authors.forename: M G
authors.initials: MG
authors.lastname: Schecter
authors.forename: M
authors.initials: M
authors.lastname: Solomon
authors.forename: D
authors.initials: D
authors.lastname: Boyer
authors.forename: C
authors.initials: C
authors.lastname: Conrad
authors.forename: T
authors.initials: T
authors.lastname: Frischer
authors.forename: J
authors.initials: J
authors.lastname: Wong
authors.forename: A
authors.initials: A
authors.lastname: Boehler
authors.forename: C
authors.initials: C
authors.lastname: Benden
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000401:mortality / Q000601:surgery*; D007223:Infant; D008168:Lung / Q000502:physiology; D016040:Lung Transplantation; D008297:Male; D012189:Retrospective Studies; D016896:Treatment Outcome