title: Primary pulmonary hypertension in non-cirrhotic portal fibrosis.
abstract: BACKGROUND
  Primary pulmonary hypertension (PPH) has been reported in association with cirrhosis and extrahepatic portal venous obstruction; reports of PPH in noncirrhotic portal fibrosis (NCPF) are few.
  
  
  OBJECTIVE
  To evaluate pulmonary arterial pressure in patients with NCPF.
  
  
  METHODS
  Twenty two patients with NCPF underwent hemodynamic studies for pulmonary arterial pressure after excluding secondary causes of pulmonary hypertension. Hemodynamic studies were carried out through the femoral route using 7F Swan-Ganz catheter. Splenoportal venography was done by percutaneous splenic puncture.
  
  
  RESULTS
  The mean pulmonary arterial pressure was 12.9 +/- 3.1 mmHg with pulmonary capillary wedge pressure of 8.3 +/- 2.1 mmHg in 20 of 22 cases; in the remaining two cases, the corresponding pressures were 30 mmHg and 28 mmHg and 13 mmHg and 12 mmHg, respectively.
  
  
  CONCLUSIONS
  Two of 22 patients with NCPF had PPH. PPH can thus develop without hepatocellular failure or recurrent embolization from portal axis thrombosis as has been described in cirrhosis.
authors.affiliation: Department of Gastroenterology, Institute of Postgraduate Medical Education and Research, Calcutta.
authors.forename: B K
authors.initials: BK
authors.lastname: De
authors.forename: A
authors.initials: A
authors.lastname: Pal
authors.forename: A
authors.initials: A
authors.lastname: Santra
authors.forename: T K
authors.initials: TK
authors.lastname: Das
authors.forename: P
authors.initials: P
authors.lastname: Biswas
authors.forename: P K
authors.initials: PK
authors.lastname: Agarwal
authors.forename: D N
authors.initials: DN
authors.lastname: Mazumder
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000000981:diagnostic imaging / Q000209:etiology / Q000503:physiopathology*; D008103:Liver Cirrhosis / Q000150:complications* / Q000000981:diagnostic imaging / Q000503:physiopathology; D008297:Male; D011172:Portography; D011446:Prospective Studies; D011669:Pulmonary Wedge Pressure; D012129:Respiratory Function Tests