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The Journal of Heart and Lung Transplantation
International Society for Heart and Lung Transplantation.

Transplant outcomes for idiopathic pulmonary hypertension in children

Published:February 05, 2019DOI:https://doi.org/10.1016/j.healun.2019.01.1314
      Idiopathic pulmonary hypertension (IPH) is an indication for lung transplantation (LTx) or combined heart‒lung transplantation (HLTx) in the pediatric population. Overall, pulmonary vascular disease is the primary diagnosis for a large minority of children listed for lung transplant, especially those <6years of age.
      • Goldfarb SB
      • Levvey BJ
      • Cherikh WS
      • et al.
      Registry of the International Society for Heart and Lung Transplantation: twentieth pediatric lung and heart‒lung transplantation report—2017; Focus theme: Allograftischemic time.
      However, the relatively low numbers of these surgical procedures performed in children limits accurate outcome data. Previously published work has included study cohorts ranging from 5 to 23 children, severely restricting the ability to predict post-thoracic transplant outcomes based on clinical characteristics.
      • Lammers A
      • Burch M
      • Benden C
      • et al.
      Lung transplantation in children with idiopathic pulmonary arterial hypertension.
      • Goldstein B
      • Sweet S
      • Jingnan M
      • Huddleston C
      • Grady RM
      Lung transplantation in children with ipiopathic pulmonary arterial hypertension: an 18-year experience.
      • Schaellibaum G
      • Lammers A
      • Faro A
      • et al.
      Bilateral lung transplantation for pediatric idiopathic pulmonary arterial hypertension.
      One-year post-transplant survival rates have ranged from 87% to 100%, with Schaellibaum et al estimating a 3-year survival of 84% and Goldstein et al reporting a 5-year survival of 61%.
      • Lammers A
      • Burch M
      • Benden C
      • et al.
      Lung transplantation in children with idiopathic pulmonary arterial hypertension.
      • Goldstein B
      • Sweet S
      • Jingnan M
      • Huddleston C
      • Grady RM
      Lung transplantation in children with ipiopathic pulmonary arterial hypertension: an 18-year experience.
      • Schaellibaum G
      • Lammers A
      • Faro A
      • et al.
      Bilateral lung transplantation for pediatric idiopathic pulmonary arterial hypertension.
      In this analysis, a national registry data set was used to evaluate outcomes for pediatric patients undergoing LTx or HLTx for IPH, and to explore clinical characteristics that may be associated with post-transplant survival.
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      References

        • Goldfarb SB
        • Levvey BJ
        • Cherikh WS
        • et al.
        Registry of the International Society for Heart and Lung Transplantation: twentieth pediatric lung and heart‒lung transplantation report—2017; Focus theme: Allograftischemic time.
        J Heart Lung Transplant. 2017; 36: 1070-1079
        • Lammers A
        • Burch M
        • Benden C
        • et al.
        Lung transplantation in children with idiopathic pulmonary arterial hypertension.
        Pediatr Pulmonol. 2010; 45: 263-269
        • Goldstein B
        • Sweet S
        • Jingnan M
        • Huddleston C
        • Grady RM
        Lung transplantation in children with ipiopathic pulmonary arterial hypertension: an 18-year experience.
        J Heart Lung Transplant. 2011; 30: 1148-1152
        • Schaellibaum G
        • Lammers A
        • Faro A
        • et al.
        Bilateral lung transplantation for pediatric idiopathic pulmonary arterial hypertension.
        Pediatr Pulmonol. 2011; 46: 1121-1127