title: Prognosis in tetralogy of Fallot with absent pulmonary valve.
abstract: BACKGROUND
  Tetralogy of Fallot with absent pulmonary valve (TF/APV) is a rare and severe congenital heart disease with high mortality. The aim of this study was to assess whether TF/APV prognosis is related to fetal and postnatal clinical course and pulmonary artery (PA) configuration.
  
  
  METHODS
  The fetal and postnatal echocardiograms and clinical outcomes of 13 patients with TF/APV (diagnosed antenatally in 9 patients and postnatally in 4) were reviewed, and divided into two groups: group A (n = 6), alive; and group D (n = 7), dead.
  
  
  RESULTS
  Fetal period: group A, polyhydramnios n = 0, hydrops fetalis (HF) n = 0, patent ductus arteriosus (PDA) n = 2; group D, polyhydramnios n = 3, HF n = 2, PDA n = 0. Postnatal period: group A, five patients underwent intracardiac repair, including one requiring artificial ventilation (AV). A further AV patient required three operations before extubation. Postoperative courses were all good. Group D, excluding the two intrauterine fetal deaths, four patients required AV (three of whom died neonatally or in early childhood) and one underwent intracardiac repair. PA configuration: all group A patients had bulbous expansion of left and right PA (clover type). Three patients in group D had bulbous expansion of main PA (balloon type).
  
  
  CONCLUSIONS
  PDA was a factor associated with good prognosis. Hydramnion and HF were factors associated with poor prognosis. Given that there was a higher rate of postnatal AV and poorer prognosis in balloon type than in clover type PA (P < 0.05), PA configuration is also considered an important factor to predict postnatal outcome in TF/APV.
authors.affiliation: Department of Pediatric Cardiology, Osaka Medical Center and Research Institute for Maternal and Child Health, Izumi City, Osaka, Japan.
authors.forename: Yukiko
authors.initials: Y
authors.lastname: Kawazu
authors.forename: Noboru
authors.initials: N
authors.lastname: Inamura
authors.forename: Ryo
authors.initials: R
authors.lastname: Ishii
authors.forename: Yoshino
authors.initials: Y
authors.lastname: Terashima
authors.forename: Yuji
authors.initials: Y
authors.lastname: Hamamichi
authors.forename: Futoshi
authors.initials: F
authors.lastname: Kayatani
authors.forename: Shigemitsu
authors.initials: S
authors.lastname: Iwai
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Kawata
authors.forename: Hidefumi
authors.initials: H
authors.lastname: Kishimoto
mesh_terms: D002648:Child; D002675:Child, Preschool; D004374:Ductus Arteriosus, Patent / Q000175:diagnosis; D004452:Echocardiography; D005260:Female; D005333:Fetus; D006801:Humans; D015160:Hydrops Fetalis / Q000175:diagnosis; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D006831:Polyhydramnios / Q000175:diagnosis; D011247:Pregnancy; D011379:Prognosis; D011664:Pulmonary Valve / Q000002:abnormalities*; D012121:Respiration, Artificial; D012307:Risk Factors; D013771:Tetralogy of Fallot / Q000175:diagnosis* / Q000628:therapy; D014057:Tomography, X-Ray Computed
keywords: absent pulmonary valve; configuration; prognosis; pulmonary artery; tetralogy of Fallot