Unexpected lethal pulmonary hypertension in a 5-year-old girl successfully treated for biliary atresia.
There appears to be a relationship between portal and pulmonary hypertension. A 5-year-old girl treated for biliary atresia developed this combination unexpectedly and died of pulmonary hypertension. Established pulmonary hypertension has a poor prognosis, which underscores the importance of early diagnosis by regular screening.
Schuijtvlot
E T
ET
Department of Pediatric Surgery, University Children's Hospital Wilhelmina, Utrecht, The Netherlands.
Bax
N M
NM
Houwen
R H
RH
Hruda
J
J
D001656:Biliary Atresia / Q000601:surgery*; D002675:Child, Preschool; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications; D006976:Hypertension, Pulmonary / Q000150:complications*; D011171:Portoenterostomy, Hepatic; D011183:Postoperative Complications / Q000209:etiology; D011379:Prognosis; D013997:Time Factors