title: Intravenous sildenafil i.v. as rescue treatment for refractory pulmonary hypertension in extremely preterm infants.
abstract: BACKGROUND
  Intravenous sildenafil treatment has recently shown promising results and good tolerability in the treatment of refractory pulmonary hypertension (PH) in term and near-term neonates, while comparable data in preterm infants are still lacking. However, for critically ill preterm infants suffering from PH refractory to conventional treatment, sildenafil may represent a last treatment resort.
  
  
  METHODS
  We reviewed the records of 6 critically ill extremely preterm infants who had suffered from PH refractory to conventional treatment and had obtained intravenous sildenafil after careful consideration as ultima ratio treatment.
  
  
  OBJECTIVE
  To describe the responses to sildenafil in terms of hemodynamic and respiratory changes during treatment and outcome.
  
  
  RESULTS
  4/6 patients showed resolution of severe PH with full reversal of ductal shunt direction into pure left-to-right shunt within 82 ± 35 h after sildenafil start. Remarkably, 2/6 patients developed pulmonary hemorrhage at a time point when significant improvement of PH had already taken place, both of them survived. Overall 4/6 patients died, two deaths were related to treatment-refractory PH.
  
  
  CONCLUSIONS
  Intravenous sildenafil treatment seems effective in improving severe PH and hemodynamic instability in extremely preterm infants with refractory PH. Pulmonary hemorrhage may represent a distinct adverse effect of sildenafil treatment in these patients, presumably due to sudden reversal of ductal shunt. Accordingly, sildenafil should be restricted to most severe and refractory cases in this population.
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Division of Neonatology, Pediatric Intensive Care and Neuropediatrics, Vienna, Austria.
authors.forename: M
authors.initials: M
authors.lastname: Steiner
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Division of Neonatology, Pediatric Intensive Care and Neuropediatrics, Vienna, Austria.
authors.forename: U
authors.initials: U
authors.lastname: Salzer
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Division of Neonatology, Pediatric Intensive Care and Neuropediatrics, Vienna, Austria.
authors.forename: S
authors.initials: S
authors.lastname: Baumgartner
authors.affiliation: Department of Epidemiology, Medical University of Vienna, Center of Public Health, Vienna, Austria.
authors.forename: T
authors.initials: T
authors.lastname: Waldhoer
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Division of Neonatology, Pediatric Intensive Care and Neuropediatrics, Vienna, Austria.
authors.forename: K
authors.initials: K
authors.lastname: Klebermass-Schrehof
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Division of Neonatology, Pediatric Intensive Care and Neuropediatrics, Vienna, Austria.
authors.forename: M
authors.initials: M
authors.lastname: Wald
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Division of Neonatology, Pediatric Intensive Care and Neuropediatrics, Vienna, Austria.
authors.forename: M
authors.initials: M
authors.lastname: Langgartner
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Division of Neonatology, Pediatric Intensive Care and Neuropediatrics, Vienna, Austria.
authors.forename: A
authors.initials: A
authors.lastname: Berger
mesh_terms: D005260:Female; D006470:Hemorrhage / Q000139:chemically induced / Q000175:diagnosis; D006801:Humans; D052577:Infant, Extremely Low Birth Weight; D007231:Infant, Newborn; D007235:Infant, Premature, Diseases / Q000175:diagnosis / Q000188:drug therapy*; D007262:Infusions, Intravenous; D007363:Intensive Care Units, Neonatal; D008171:Lung Diseases / Q000139:chemically induced / Q000175:diagnosis; D008297:Male; D010547:Persistent Fetal Circulation Syndrome / Q000175:diagnosis / Q000188:drug therapy*; D010879:Piperazines / Q000008:administration & dosage* / Q000009:adverse effects; D011669:Pulmonary Wedge Pressure / Q000187:drug effects; D011687:Purines / Q000008:administration & dosage / Q000009:adverse effects; D012189:Retrospective Studies; D000068677:Sildenafil Citrate; D013449:Sulfonamides / Q000008:administration & dosage* / Q000009:adverse effects; D014665:Vasodilator Agents / Q000008:administration & dosage* / Q000009:adverse effects
chemical_list: D010879:Piperazines; D011687:Purines; D013449:Sulfonamides; D014665:Vasodilator Agents; D000068677:Sildenafil Citrate