title: Persistent pulmonary hypertension of the newborn with transposition of the great arteries.
abstract: BACKGROUND
  Persistent pulmonary hypertension of the newborn (PPHN) in patients with transposition of the great arteries (TGA) is reported to be a high-risk and often therapy-resistant condition, associated with a high mortality. However, data on its incidence and prognosis are scarce and originate mostly from the era before introduction of inhaled nitric oxide (iNO) therapy for PPHN.
  
  
  METHODS
  This is a retrospective study of consecutive newborns with TGA, admitted to a tertiary cardiac and neonatal intensive unit over a 10-year period. In this period, iNO therapy was available.
  
  
  RESULTS
  Fourteen out of 112 patients with TGA (12.5%) presented with associated PPHN. The PPHN occurred more frequently in patients with TGA and intact ventricular septum (IVS) compared with those with TGA and ventricular septal defect (13 out of 83 patients versus one out of 29 patients, respectively; p = 0.06, Fisher exact test). Of those newborns, six presented with severe PPHN, whereas eight presented with mild-to-moderate PPHN. Despite currently available treatment modalities, including iNO, four out of 14 patients died before corrective surgical procedures were considered to be an option (TGA/PPHN preoperative mortality 28.6%). These included three out of six patients (50%) with severe PPHN and one out of eight (12.5%) with mild-to-moderate PPHN.
  
  
  CONCLUSIONS
  The combination of TGA with PPHN is a serious and often fatal condition. It may jeopardize the usually favorable outcome of newborns with TGA. Despite the introduction of iNO therapy, the combination of TGA and PPHN remains a condition with unacceptable high mortality (in our series). Additional treatment strategies need to be investigated.
authors: Department of Paediatric Cardiology, Beatrix Children's Hospital, Groningen, The Netherlands. m.t.r.roofthooft@bkk.umcg.nl
authors: Marcus T R
authors: MT
authors: Roofthooft
authors: Klasina A
authors: KA
authors: Bergman
authors: Tjalling W
authors: TW
authors: Waterbolk
authors: Tjark
authors: T
authors: Ebels
authors: Beatrijs
authors: B
authors: Bartelds
authors: Rolf M F
authors: RM
authors: Berger
mesh_terms: D006348:Cardiac Surgical Procedures / Q000379:methods / Q000401:mortality; D002423:Cause of Death; D015331:Cohort Studies; D003131:Combined Modality Therapy; D015897:Comorbidity; D005260:Female; D006801:Humans; D015994:Incidence; D007231:Infant, Newborn; D007363:Intensive Care Units, Neonatal; D008297:Male; D010547:Persistent Fetal Circulation Syndrome / Q000175:diagnosis / Q000453:epidemiology* / Q000628:therapy; D011336:Probability; D011379:Prognosis; D012189:Retrospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D018709:Statistics, Nonparametric; D016019:Survival Analysis; D014188:Transposition of Great Vessels / Q000175:diagnosis / Q000453:epidemiology* / Q000628:therapy