title: Inhaled iloprost plus oral sildenafil in patients with severe pulmonary arterial hypertension delays the need for lung transplantation.
abstract: BACKGROUND
  Accepted treatment for severe pulmonary arterial hypertension (PAH) includes intravenous epoprostenol and lung transplantation (LT). Inhaled iloprost plus oral sildenafil (Ilo-Sil) is an alternative strategy that may also delay the need for LT.
  
  
  METHODS
  This was a long-term descriptive study in eight patients with PAH functional class (FC) IV with right heart failure, four of them potential candidates for LT, who were treated with Ilo-Sil as an alternative to epoprostenol.
  
  
  RESULTS
  At the start of the study, patients (seven women; mean age, 43.8 [range, 34-66] years) were in FC IV and unable to perform the 6-minute walk test. Mean cardiac index was 1.9 (range, 1.4-2.1) L/min/m(2). Treatment with Ilo-Sil provoked a rapid and sustained improvement; mean walking distance at 3 months was 322 ± 90 m and no patient remained in FC IV. Survival at 1 and 5 years was 100% and 75%, respectively. Of the four potential LT candidates, one underwent transplantation after 6.8 years and one died after 1.2 years.
  
  
  CONCLUSIONS
  These results suggest that therapy with Ilo-Sil represents an acceptable alternative in patients with severe and unstable PAH.
authors: Respiratory Department, Institute of Research, Hospital Universitari Vall d'Hebron, Universitat Autònoma of Barcelona, Barcelona, Spain.
authors: M
authors: M
authors: Lopez-Meseguer
authors: C
authors: C
authors: Berastegui
authors: V
authors: V
authors: Monforte
authors: C
authors: C
authors: Bravo
authors: E
authors: E
authors: Domingo
authors: A
authors: A
authors: Roman
mesh_terms: D000280:Administration, Inhalation; D000284:Administration, Oral; D000328:Adult; D000368:Aged; D000959:Antihypertensive Agents / Q000008:administration & dosage*; D062186:Arterial Pressure / Q000187:drug effects*; D004359:Drug Therapy, Combination; D017079:Exercise Tolerance / Q000187:drug effects; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy* / Q000401:mortality / Q000503:physiopathology / Q000601:surgery; D016285:Iloprost / Q000008:administration & dosage*; D008137:Longitudinal Studies; D016040:Lung Transplantation; D008297:Male; D008875:Middle Aged; D058986:Phosphodiesterase 5 Inhibitors / Q000008:administration & dosage*; D010879:Piperazines / Q000008:administration & dosage*; D011651:Pulmonary Artery / Q000187:drug effects* / Q000503:physiopathology / Q000601:surgery; D011687:Purines / Q000008:administration & dosage; D020127:Recovery of Function; D012720:Severity of Illness Index; D000068677:Sildenafil Citrate; D013450:Sulfones / Q000008:administration & dosage*; D013997:Time Factors; D061665:Time-to-Treatment; D016896:Treatment Outcome; D014665:Vasodilator Agents / Q000008:administration & dosage*
chemical_list: D000959:Antihypertensive Agents; D058986:Phosphodiesterase 5 Inhibitors; D010879:Piperazines; D011687:Purines; D013450:Sulfones; D014665:Vasodilator Agents; D000068677:Sildenafil Citrate; D016285:Iloprost