title: Oral sildenafil for persistent pulmonary hypertension early after congenital cardiac surgery in children.
abstract: OBJECTIVE
  Sildenafil is a strong pulmonary vasodilator that increases the intracellular cyclic guanosine monophosphate concentration through inhibition of phosphodiesterase-5. We assessed the benefit of oral sildenafil for persistent pulmonary hypertension early after congenital cardiac surgery in paediatric patients.
  
  
  METHODS
  Sildenafil was administered at a starting dose of 0.5 mg kg(-1) following admission to the intensive care unit. With careful monitoring of haemodynamics, the dose was increased stepwise by 0.5 mg kg(-1) every 4-6 h up to a maximum of 2 mg kg(-1). After successful weaning from a ventilator and from other vasodilators, sildenafil was gradually discontinued over the next 5-7 days.
  
  
  RESULTS
  A retrospective review of medical records showed an age distribution of <1 month (n=26), > or = 1-<6 months (n=36), > or = 6-<12 months (n=19), 1-3 years (n=8), 4-9 years (n=9) and >10 years (n=2) at the time of surgery. The surgeries were performed for ventricular septal defect closure (n=17), arterial switch (n=30), truncus arteriosus repair (n=10), complete atrioventricular septal defect repair (n=12), total anomalous venous drainage repair (n=9), and other open-heart surgery (n=22). The aforementioned concomitant inhaled nitrous oxide treatment was performed in 66 patients. Pulmonary arterial pressure decreased in 28, was unchanged in five and elevated in one patient out of the total of 34 cases for which data from continuous pressure monitoring were available. Bosentan was added in three cases with persistent symptoms due to pulmonary hypertension despite sildenafil treatment. After sildenafil administration, modest oxygen desaturation occurred in seven cases, but no 'rebound' pulmonary hypertension occurred. There were no significant adverse events during sildenafil treatment.
  
  
  CONCLUSIONS
  Our results suggest that oral sildenafil is a safe and effective alternate for persistent pulmonary hypertension following congenital heart surgery in children.
authors.affiliation: Department of Cardiovascular Surgery, Osaka Medical College Hospital, Takatsuki, Osaka, Japan. snemoto@poh.osaka-med.ac.jp
authors.forename: Shintaro
authors.initials: S
authors.lastname: Nemoto
authors.forename: Tomoyasu
authors.initials: T
authors.lastname: Sasaki
authors.forename: Hideki
authors.initials: H
authors.lastname: Ozawa
authors.forename: Takahiro
authors.initials: T
authors.lastname: Katsumata
authors.forename: Kanta
authors.initials: K
authors.lastname: Kishi
authors.forename: Kenichi
authors.initials: K
authors.lastname: Okumura
authors.forename: Yasuhiko
authors.initials: Y
authors.lastname: Mori
authors.forename: Osamu
authors.initials: O
authors.lastname: Umegaki
mesh_terms: D000284:Administration, Oral; D017677:Age Distribution; D002648:Child; D002675:Child, Preschool; D004334:Drug Administration Schedule; D006330:Heart Defects, Congenital / Q000601:surgery*; D006439:Hemodynamics / Q000187:drug effects; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000503:physiopathology; D007223:Infant; D007231:Infant, Newborn; D010101:Oxygen Consumption / Q000187:drug effects; D058986:Phosphodiesterase 5 Inhibitors / Q000009:adverse effects / Q000494:pharmacology / Q000627:therapeutic use; D010879:Piperazines / Q000009:adverse effects / Q000494:pharmacology / Q000627:therapeutic use*; D011182:Postoperative Care / Q000379:methods; D011183:Postoperative Complications / Q000188:drug therapy* / Q000503:physiopathology; D011652:Pulmonary Circulation / Q000187:drug effects; D011687:Purines / Q000009:adverse effects / Q000494:pharmacology / Q000627:therapeutic use; D000068677:Sildenafil Citrate; D013450:Sulfones / Q000009:adverse effects / Q000494:pharmacology / Q000627:therapeutic use*; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000009:adverse effects / Q000494:pharmacology / Q000627:therapeutic use*
chemical_list: D058986:Phosphodiesterase 5 Inhibitors; D010879:Piperazines; D011687:Purines; D013450:Sulfones; D014665:Vasodilator Agents; D000068677:Sildenafil Citrate