Long-term outcomes after infant lung transplantation for surfactant protein B deficiency related to other causes of respiratory failure.
OBJECTIVE
To determine if the outcomes of lung transplantation for infants with surfactant protein-B (SP-B) deficiency are unique.


METHODS
From a prospective analysis to identify infants with genetic causes of surfactant deficiency, we identified 33 SP-B-deficient infants from 1993 to 2005, and, among those undergoing lung transplantation (n = 13), compared their survival, pulmonary function, and developmental progress with infants who underwent transplantation at <1 year of age for parenchymal lung disease (n = 13) or pulmonary vascular disease (n = 11).


RESULTS
Five-year survival rates ( approximately 50%, P = .3) and causes of death were similar for all three groups once the infants underwent transplantation. However, significant pretransplantation mortality decreased 5-year survival from listing to approximately 30% (P = .17). Pulmonary function, development of bronchiolitis obliterans, and school readiness were similar among the three groups. We detected anti SP-B antibody in serum of 3 of 7 SP-B-deficient infants and none of 7 SP-B-sufficient infants but could not identify any associated adverse outcomes.


CONCLUSIONS
Long-term outcomes after infant lung transplantation for SP-B-deficient infants are similar to those of infants transplanted for other indications. These outcomes are important considerations in deciding to pursue lung transplantation for infants with disorders of alveolar homeostasis.
Palomar
Lisanne M
LM
Edward Mallinckrodt Department of Pediatrics, Washington University, St Louis, Missouri 63110, USA.
Nogee
Lawrence M
LM
Sweet
Stuart C
SC
Huddleston
Charles B
CB
Cole
F Sessions
FS
Hamvas
Aaron
A
D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D016040:Lung Transplantation; D008297:Male; D011446:Prospective Studies; D037701:Pulmonary Surfactant-Associated Protein B / Q000172:deficiency*; D012131:Respiratory Insufficiency / Q000209:etiology* / Q000601:surgery*; D013997:Time Factors; D016896:Treatment Outcome
D037701:Pulmonary Surfactant-Associated Protein B