title: Prevalence of spontaneous portosystemic shunts in patients with portopulmonary hypertension and effect on treatment.
abstract: OBJECTIVE
  We documented the frequency of large spontaneous portosystemic shunts in patients with moderate or severe portopulmonary hypertension (POPH) and determined the association between large shunts and response to treatment.
  
  
  METHODS
  We performed a retrospective case-control study of data from patients with mild (mean pulmonary artery pressure [MPAP], 25-35 mm Hg; n = 18), moderate (MPAP, 35-50 mm Hg; n = 45), and severe POPH (MPAP, >50 mm Hg; n = 16). Data were compared with those from controls (normal echocardiography with estimated right ventricular systolic pressure, <35 mm Hg; n = 122). Spontaneous portosystemic shunts greater than 10 mm in diameter, identified by computed tomography or magnetic resonance, were classified as large. Response to treatment at 6 months was defined by right ventricular systolic pressure or MPAP as significant (<35 mm Hg), partial (35-50 mm Hg), or no response (>50 mm Hg).
  
  
  RESULTS
  The frequency of spontaneous shunts did not differ significantly between groups of subjects with severe (n = 14 of 16), moderate (n = 38 of 45), or mild POPH (n = 11 of 18) or normal echocardiograms (controls, n = 86 of 122) (P = .77). Large shunts were associated with severe (14 of 16) and moderate POPH (32 of 45), compared with mild POPH (6 of 18) or controls (30 of 122) (P < .01). In 13 patients with severe POPH, large shunts were associated with lack of response to treatment in 90% (8 of 9) or partial response in 50% (2 of 4). Among 27 patients with moderate POPH, large shunts were associated with no response to treatment in 13 of 19 (68%) and a partial response in 2 of 6 (33%).
  
  
  CONCLUSIONS
  Large spontaneous portosystemic shunts are associated significantly with moderate and severe POPH, and with lack of response to treatment.
authors: Advanced Liver Diseases Study Group, Mayo Clinic, Rochester, Minnesota 55905, USA.
authors: Jayant A
authors: JA
authors: Talwalkar
authors: Karen L
authors: KL
authors: Swanson
authors: Michael J
authors: MJ
authors: Krowka
authors: James C
authors: JC
authors: Andrews
authors: Patrick S
authors: PS
authors: Kamath
mesh_terms: D000328:Adult; D000368:Aged; D000959:Antihypertensive Agents / Q000627:therapeutic use; D000077300:Bosentan; D016022:Case-Control Studies; D002648:Child; D004359:Drug Therapy, Combination; D011464:Epoprostenol / Q000627:therapeutic use; D005260:Female; D006801:Humans; D006975:Hypertension, Portal / Q000188:drug therapy* / Q000503:physiopathology*; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000503:physiopathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D008643:Mesentery / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology*; D008875:Middle Aged; D010879:Piperazines / Q000627:therapeutic use; D011687:Purines / Q000627:therapeutic use; D012039:Regional Blood Flow / Q000502:physiology; D012082:Renal Veins / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology*; D012189:Retrospective Studies; D012720:Severity of Illness Index; D000068677:Sildenafil Citrate; D013162:Splenic Vein / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology*; D013449:Sulfonamides / Q000627:therapeutic use; D013450:Sulfones / Q000627:therapeutic use; D036542:Tomography, Spiral Computed; D016896:Treatment Outcome; D014682:Vena Cava, Inferior / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology*
chemical_list: D000959:Antihypertensive Agents; D010879:Piperazines; D011687:Purines; D013449:Sulfonamides; D013450:Sulfones; D000068677:Sildenafil Citrate; D011464:Epoprostenol; D000077300:Bosentan