Bilateral lung transplantation for pediatric idiopathic pulmonary arterial hypertension: a multi-center experience

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.