title: [Disproportionate pulmonary arterial hypertension and lung respiratory diseases: distinctive clinical, hemodynamic and prognosis of patients versus primary pulmonary arterial hypertension].
abstract: OBJECTIVE
  To characterize and compare patients with disproportionate PH versus patients with primary pulmonary arterial hypertension (PAH).
  
  
  METHODS
  All patients referred to our cardiology unit for echocardiography from November 2006 to May 2008 and who have been followed by our pneumologist were screened for severe PH (i.e mean arterial pulmonary pressure>35-40 mmHg at rest). Patients were excluded if a factor that could influence pulmonary hemodynamics was present. We investigated these patients by pulmonary function tests, echocardiography and right heart catheterisation.
  
  
  RESULTS
  We reported 16 cases of severe PH in stable patients (n=8, chronic obstructive pulmonary disease-emphysema) and 13 patients with PAH. Our findings suggest that the patients with disproportionate PH had right heart dysfunction similar to that observed in PAH. But their outcomes were more severe. It seemed that specific vasodilatator therapy was not efficient.
authors.affiliation: Service de cardiologie, centre hospitalier Princesse-Grace, Monaco, Monaco. th.hugues@yahoo.fr
authors.forename: T
authors.initials: T
authors.lastname: Hugues
authors.forename: F
authors.initials: F
authors.lastname: Lemoigne
authors.forename: F
authors.initials: F
authors.lastname: Macone
authors.forename: P
authors.initials: P
authors.lastname: Cerboni
authors.forename: P
authors.initials: P
authors.lastname: Gibelin
mesh_terms: D000368:Aged; D004452:Echocardiography; D065627:Familial Primary Pulmonary Hypertension; D005260:Female; D006439:Hemodynamics; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000000981:diagnostic imaging / Q000503:physiopathology*; D008171:Lung Diseases / Q000150:complications* / Q000000981:diagnostic imaging / Q000503:physiopathology*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011446:Prospective Studies; D012120:Respiration Disorders / Q000150:complications / Q000000981:diagnostic imaging / Q000503:physiopathology*