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Lung transplantation in children with idiopathic pulmonary arterial hypertension.

Lammers, AE; Burch, M; Benden, C; Elliott, MJ; Rees, P; Haworth, SG; Aurora, P; (2010) Lung transplantation in children with idiopathic pulmonary arterial hypertension. Pediatr Pulmonol , 45 (3) pp. 263-269. 10.1002/ppul.21168.

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Abstract

BACKGROUND: Despite improved medical therapy, transplantation (Tx) represents the only option for end-stage pulmonary vascular disease. METHODS: Clinical data of children with idiopathic pulmonary arterial hypertension (IPAH) referred for Tx assessment between January 2002 and June 2007 were related to listing decision and outcome. RESULTS: Seven of the 14 children assessed for Tx were listed. Five were transplanted (lung Tx, n = 3; heart-lung Tx, n = 2) and two died on the waiting list. Mean age at diagnosis was 3.7 (0.4-9.5) years. Time from diagnosis to listing was 3.6 years (range 1.4-9.3). Children listed were in a worse functional class (WHO 3.5 vs. 2.5; P = 0.0006), had a lower SpO(2) on exercise (76.5% vs. 89%; P = 0.0001) and a shorter 6-min walk distance (154 m vs. 330 m; P < 0.01) than those not listed. Right ventricular function was worse in those listed (P = 0.03), as was pulmonary vascular resistance index (PVRI) on vasodilator testing (34 U m(2) vs. 14.6 U m(2); P = 0.03). Age at diagnosis and at assessment, weight, height, mean pulmonary artery pressure, baseline PVRI, B-type natriuretic peptide, spirometry and resting-SO(2) did not differ between the two groups. For the five children transplanted, median waiting time was 81 days. Age at Tx was 5.4 years. After 2.8 years all transplanted children are alive with a good functional outcome. Two patients died on the active waiting list. All children considered too well for listing are still alive and stable on treatment. CONCLUSIONS: Outcome after transplantation in children with IPAH has been encouraging. Defining listing criteria for these patients remains a challenge.

Type: Article
Title: Lung transplantation in children with idiopathic pulmonary arterial hypertension.
Location: United States
DOI: 10.1002/ppul.21168
Keywords: Adolescent, Child, Child, Preschool, Female, Follow-Up Studies, Humans, Hypertension, Pulmonary, Infant, Lung Transplantation, Male, Patient Selection, Recovery of Function, Retrospective Studies, Survival Analysis
UCL classification: UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Pop Health Sciences > Institute of Cardiovascular Science
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Pop Health Sciences > UCL GOS Institute of Child Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Pop Health Sciences > UCL GOS Institute of Child Health > ICH Infect, Imm, Infla. and Physio Med
URI: http://discovery.ucl.ac.uk/id/eprint/1392834
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