title: Effect of PAH specific therapy on pulmonary hemodynamics and six-minute walk distance in portopulmonary hypertension: a systematic review and meta-analysis.
abstract: BACKGROUND
  Little is known about the effect of pulmonary arterial hypertension (PAH) specific therapy on pulmonary hemodynamics and exercise capacity in patients with portopulmonary hypertension (PoPH) because such patients are usually excluded from randomized clinical trials (RCT) of such therapy.
  
  
  METHODS
  We searched PUBMED using the terms "(Therapy/Broad (filter)) AND (portopulmonary hypertension)." We included studies that met the following criteria: ≥5 patients, AND PoPH confirmed by right heart catheterization (RHC), AND follow-up RHC data, AND/OR baseline and follow-up 6MWD available.
  
  
  RESULTS
  12 studies met our inclusion criteria. None was a RCT. The baseline mPAP was 48.6 ± 4.4 mmHg, cardiac output (CO) 5.6 ± 0.9 L/min, and pulmonary vascular resistance (PVR) 668.6 ± 219.1 dynes.sec/cm(5). The baseline 6MWD was 348.2 ± 35.6 meters. The use of PAH specific therapy improved mPAP by 7.54 mmHg (95% CI 10.2 to 4.9), CO by 1.77 L/min (95% CI 1.1 to 2.4), and PVR by 253 dynes.sec/cm(5) (95% CI 291.4 to 214.6) (n = 135) and 6MWD by 61.8 meters (95% CI 47.5 to 76) (n = 122).
  
  
  CONCLUSIONS
  The use of PAH specific therapy in PoPH results in significant improvement in both pulmonary hemodynamics and 6MWD.
authors.affiliation: Division of Pulmonary, Critical Care and Sleep Medicine, University of Florida, Jacksonville, FL 32209, USA.
authors.forename: Muhammad
authors.initials: M
authors.lastname: Faisal
authors.affiliation: Division of Pulmonary, Critical Care and Sleep Medicine, University of Florida, Jacksonville, FL 32209, USA.
authors.forename: Furqan
authors.initials: F
authors.lastname: Siddiqi
authors.affiliation: Department of Medicine, University of Florida, Jacksonville, FL 32209, USA.
authors.forename: Ahmad
authors.initials: A
authors.lastname: Alkaddour
authors.affiliation: Division of Pulmonary, Critical Care and Sleep Medicine, University of Florida, Jacksonville, FL 32209, USA.
authors.forename: Abubakr A
authors.initials: AA
authors.lastname: Bajwa
authors.affiliation: Division of Pulmonary, Critical Care and Sleep Medicine, University of Florida, Jacksonville, FL 32209, USA.
authors.forename: Adil
authors.initials: A
authors.lastname: Shujaat
mesh_terms: D005260:Female; D006439:Hemodynamics / Q000502:physiology; D006801:Humans; D006975:Hypertension, Portal / Q000150:complications / Q000503:physiopathology*; D006976:Hypertension, Pulmonary / Q000150:complications / Q000503:physiopathology / Q000628:therapy*; D008168:Lung / Q000098:blood supply; D008297:Male; D008875:Middle Aged; D010807:Physical Endurance / Q000502:physiology; D016138:Walking / Q000502:physiology