Alveolar capillary dysplasia: a cause of persistent pulmonary hypertension of the newborn.
The term alveolar capillary dysplasia refers to complex vascular abnormalities which have recently been identified in some infants with persistent pulmonary hypertension. We report four cases admitted to our institution for severe pulmonary hypertension unresponsive to maximal cardiorespiratory support, including high-frequency ventilation, inhaled nitric oxide and extracorporeal membrane oxygenation. The four infants died of refractory hypoxaemia. The diagnosis of alveolar capillary dysplasia was established by necropsy. We have used these cases as an opportunity for a thorough review of the literature containing comments regarding aetiology, pathophysiology, clinical presentation, associated malformations and treatment trials.


CONCLUSIONS
alveolar capillary dysplasia should be ruled out in all newborn infants presenting severe idiopathic pulmonary hypertension associated with malformations. Open lung biopsy may prevent from using costly, invasive and probably ineffective procedures such as extracorporeal membrane oxygenation.
Alameh
Joumana
J
Department of Neonatology, Service de Médecine Néonatale, Hôpital Jeanne de Flandre, CHRU de Lille, Lille cédex 59037, France.
Bachiri
Ahmed
A
Devisme
Louise
L
Truffert
Patrick
P
Rakza
Thameur
T
Riou
Yvon
Y
Manouvrier
Sylvie
S
Lequien
Pierre
P
Storme
Laurent
L
D002196:Capillaries / Q000002:abnormalities; D015199:Extracorporeal Membrane Oxygenation; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D010547:Persistent Fetal Circulation Syndrome / Q000209:etiology* / Q000628:therapy; D011650:Pulmonary Alveoli / Q000098:blood supply*