Lung transplantation in children with idiopathic pulmonary arterial hypertension.
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.
Great Ormond Street Hospital for Children, Great Ormond Street, London WC1N 3JH, UK. astridlammers@gmx.de
Astrid E
AE
Lammers
Michael
M
Burch
Christian
C
Benden
Martin J
MJ
Elliott
Philip
P
Rees
Sheila G
SG
Haworth
Paul
P
Aurora
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000601:surgery*; D007223:Infant; D016040:Lung Transplantation; D008297:Male; D018579:Patient Selection; D020127:Recovery of Function; D012189:Retrospective Studies; D016019:Survival Analysis