title: Prenatal pulmonary hypertension index: novel prenatal predictor of severe postnatal pulmonary artery hypertension in antenatally diagnosed congenital diaphragmatic hernia.
abstract: OBJECTIVE
  This study aim to assess the potential of prenatal predictors of postnatal severe pulmonary artery hypertension (PAH) in isolated left congenital diaphragmatic hernia (CDH) and to define a new prenatal pulmonary hypertension index (PPHI).
  
  
  METHODS
  A retrospective chart review of CDH patients between May 2005 and October 2008 was conducted. Ten patients with systemic/suprasystemic and 9 patients with subsystemic pulmonary hypertension at 3 weeks of age were identified. Diameters of the right pulmonary artery, left pulmonary artery (LPA(d)), aorta, and the length of vermis of the cerebellum were obtained from prenatal magnetic resonance imaging to calculate the PPHI [=(LPA(d)/length of vermis of the cerebellum) x 10] and the modified McGoon index (MGI) [=(diameter of the right pulmonary artery + LPA(d))/diameter of aorta]. Prenatal pulmonary hypertension index and MGI were compared with lung-to-head ratio, percent predicted lung volume, and total lung volume for pulmonary hypertension and survival.
  
  
  RESULTS
  The PPHI and MGI had a significant, negative correlation with pulmonary hypertension (r = -0.61, P = .005, and r = -0.72, P < .005, respectively). The PPHI and MGI are significantly lower in the systemic/suprasystemic PAH group compared with the subsystemic PAH group (1.11 +/- 0.32 versus 1.63 +/- 0.28, P = .004, and 0.71 +/- 0.15 versus 1.05 +/- 0.11, P < .001, respectively). There were no significant differences between the groups comparing the lung-to-head ratio, percent predicted lung volume, and total lung volume.
  
  
  CONCLUSIONS
  Both PPHI and MGI accurately predict the severity of postnatal PAH in isolated left CDH.
authors.affiliation: Division of Pediatric and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH 45229, USA.
authors.forename: Jose F
authors.initials: JF
authors.lastname: Vuletin
authors.forename: Foong-Yen
authors.initials: FY
authors.lastname: Lim
authors.forename: James
authors.initials: J
authors.lastname: Cnota
authors.forename: Beth
authors.initials: B
authors.lastname: Kline-Fath
authors.forename: Shelia
authors.initials: S
authors.lastname: Salisbury
authors.forename: Beth
authors.initials: B
authors.lastname: Haberman
authors.forename: Paul
authors.initials: P
authors.lastname: Kingma
authors.forename: Jason
authors.initials: J
authors.lastname: Frischer
authors.forename: Timothy
authors.initials: T
authors.lastname: Crombleholme
mesh_terms: D000465:Algorithms; D001837:Body Weights and Measures; D017352:Echo-Planar Imaging; D004452:Echocardiography; D005260:Female; D006548:Hernia, Diaphragmatic / Q000175:diagnosis; D065630:Hernias, Diaphragmatic, Congenital; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis*; D007231:Infant, Newborn; D016015:Logistic Models; D015999:Multivariate Analysis; D010865:Pilot Projects; D011247:Pregnancy; D011296:Prenatal Diagnosis; D012189:Retrospective Studies; D012680:Sensitivity and Specificity; D012720:Severity of Illness Index
references.citation: J Pediatr Surg. 2007 Jan;42(1):193-7
references.pmid: 17208564
references.citation: J Pediatr Surg. 1996 Jan;31(1):148-51; discussion 151-2
references.pmid: 8632269
references.citation: J Am Soc Echocardiogr. 2006 Dec;19(12):1413-30
references.pmid: 17138024
references.citation: Am J Obstet Gynecol. 2006 Aug;195(2):470-7
references.pmid: 16777047
references.citation: Prenat Diagn. 2008 Jul;28(7):592-603
references.pmid: 18551724
references.citation: Semin Fetal Neonatal Med. 2009 Feb;14(1):8-13
references.pmid: 18845492
references.citation: J Pediatr Surg. 2008 Apr;43(4):606-11
references.pmid: 18405704
references.citation: J Pediatr Surg. 2006 May;41(5):888-92
references.pmid: 16677876
references.citation: J Pediatr Surg. 2004 Mar;39(3):307-12; discussion 307-12
references.pmid: 15017543
references.citation: J Pediatr Surg. 2006 Jan;41(1):25-8; discussion 25-8
references.pmid: 16410102
references.citation: Semin Pediatr Surg. 1996 Nov;5(4):234-42
references.pmid: 8936652
references.citation: Eur J Pharmacol. 2000 Dec 27;410(2-3):107-120
references.pmid: 11134663