title: Persistent Pulmonary Hypertension of the Newborn with D-transposition of the Great Arteries: Management and Prognosis.
abstract: OBJECTIVE
  There is a paucity of data on clinical correlates and outcomes of pulmonary hypertension (PH) in patients with D-transposition of the great arteries (D-TGA) in the era of inhaled nitric oxide (iNO) and extracorporeal membrane oxygenation (ECMO). Our objective was to compare clinical characteristics and outcomes of infants with D-TGA with and without PH, defined as hypoxemia that required iNO and/or ECMO.
  
  
  METHODS
  We undertook a single-center retrospective chart review involving infants with gestational age ≥32 weeks with D-TGA who, underwent arterial switch operation over a 12-year period. Demographic and clinical data, details of the repair and postoperative complications were abstracted.
  
  
  RESULTS
  Our cohort (n = 93), 61 (66%) of whom were males, had a mean (SD) gestational age and birth weight of 38.7 (1.8) weeks and 3.2 (0.6) kg, respectively. PH requiring iNO and/or ECMO was noted in 20 (21.5%) infants. Infants with PH had significantly lower birth weight [2.8 (0.56) vs. 3.33 (0.61)] and gestational age [37.7 (2.1) vs. 38.9 (1.7)] than those without PH. Rates of postoperative complications (duration of pressors, sedative medicaiton and duration of hospital stay, and mechanical ventilation were higher in the group with PH. Of the five (5.4%) infants who died, four received iNO and ECMO. Death or postoperative complications tended to be associated with lower gestational age [OR 0.689; 95% CI: 0.469-1.012, P = 0.058] but not with D-TGA category or bypass duration.
  
  
  CONCLUSIONS
  Despite aggressive treatment with iNO and ECMO, the coexistence of PH in this population is associated with higher rates of mortality and postoperative complications. Our results also suggest that an early term birth may be associated with PH in infants with D-TGA.
authors.affiliation: Department of Pediatrics, Wayne State University, Children's Hospital of Michigan, Detroit, Mich, USA.
authors.forename: Salaam
authors.initials: S
authors.lastname: Sallaam
authors.affiliation: Department of Pediatrics, Wayne State University, Children's Hospital of Michigan, Detroit, Mich, USA.
authors.forename: Girija
authors.initials: G
authors.lastname: Natarajan
authors.affiliation: Department of Pediatrics, Wayne State University, Children's Hospital of Michigan, Detroit, Mich, USA.
authors.forename: Sanjeev
authors.initials: S
authors.lastname: Aggarwal
mesh_terms: D000280:Administration, Inhalation; D006348:Cardiac Surgical Procedures / Q000009:adverse effects / Q000401:mortality; D003131:Combined Modality Therapy; D015199:Extracorporeal Membrane Oxygenation / Q000009:adverse effects / Q000401:mortality; D005260:Female; D005865:Gestational Age; D017052:Hospital Mortality; D006801:Humans; D007223:Infant; D007226:Infant Mortality; D007231:Infant, Newborn; D007234:Infant, Premature; D008297:Male; D008824:Michigan; D009569:Nitric Oxide / Q000008:administration & dosage* / Q000009:adverse effects; D016017:Odds Ratio; D010547:Persistent Fetal Circulation Syndrome / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000628:therapy*; D011183:Postoperative Complications / Q000209:etiology / Q000401:mortality / Q000628:therapy; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors; D014188:Transposition of Great Vessels / Q000175:diagnosis / Q000401:mortality / Q000503:physiopathology / Q000601:surgery*; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000008:administration & dosage* / Q000009:adverse effects
chemical_list: D014665:Vasodilator Agents; D009569:Nitric Oxide
keywords: Outcome; Pulmonary Hypertension; Transposition of Great Arteries