title: Effect of beraprost sodium on pulmonary vascular resistance in candidates for a Fontan procedure: a preliminary study.
abstract: OBJECTIVE
  Although long-term prostacyclin(PGI2) therapy in patients with severe pulmonary hypertension (PH)reduces pulmonary vascular resistance (PVR), there have been no reports on its therapeutic effects in patients with mild PH. We investigated the chronic effect of beraprost sodium (BPS), an oral PGI2 analog, in children with mild PH.
  
  
  METHODS
  We studied 20 patients who were destined for a Fontan procedure with a mean pulmonary arterial pressure(PAP) of>20 mmHg and/or PVR of>3.0 Wood units. Both the PAP and the PVR in these cases were too high for patients to undergo a successful Fontan procedure. Seven patients received BPS (PG group) and 13 did not (control group). All patients underwent repeat cardiac catheterization to examine pulmonary hemodynamics.
  
  
  RESULTS
  In the PG group, the pulmonary-to-systemic flow ratio (Qp/Qs) did not change after BPS administration(1.1 +/- 0.6 vs 1.3 +/- 0.9);however, the mean PAP decreased significantly (25.3 +/- 8.2 vs 19.9 +/- 6.5 mmHg; P < 0.05),as did PVR (3.7 +/- 1.3 vs 2.3 +/- 0.9 Wood units; P < 0.05), whereas the pulmonary artery (PA) index increased significantly (312 +/- 136 vs 375 +/- 165; P < 0.05). In the control group, the mean PAP decreased significantly (24.9 +/- 4.7 vs 19.8 +/- 6.3 mmHg; P < 0.05)and the PA index increased significantly (295 +/- 72 vs 362 +/- 114; P < 0.05). No significant changes in Qp/Qs (1.5 +/- 0.8 vs 1.4 +/- 0.6)or PVR (2.9 +/- 1.3 vs 2.5 +/- 0.8 Wood units) were observed.
  
  
  CONCLUSIONS
  We conclude that long-term BPS administration probably reduces PVR in potential candidates for a Fontan procedure with mild PH. This treatment would reduce the risks associated with the Fontan procedure and would also improve its outcome.
authors.affiliation: Pediatric Cardiology, The Heart Institute of Japan, Tokyo Women's Medical University, Tokyo, Japan. bigkaz@mua.biglobe.ne.jp
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Takahashi
authors.forename: Yoshiki
authors.initials: Y
authors.lastname: Mori
authors.forename: Hideshi
authors.initials: H
authors.lastname: Yamamura
authors.forename: Toshio
authors.initials: T
authors.lastname: Nakanishi
authors.forename: Makoto
authors.initials: M
authors.lastname: Nakazawa
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D000075202:Contraindications; D011464:Epoprostenol / Q000031:analogs & derivatives* / Q000627:therapeutic use*; D005260:Female; D018729:Fontan Procedure; D006330:Heart Defects, Congenital / Q000150:complications* / Q000601:surgery; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology / Q000503:physiopathology; D008297:Male; D011652:Pulmonary Circulation / Q000187:drug effects*; D014655:Vascular Resistance / Q000187:drug effects*; D014665:Vasodilator Agents / Q000627:therapeutic use*
chemical_list: D014665:Vasodilator Agents; C048081:beraprost; D011464:Epoprostenol