title: Echocardiography may help detect pulmonary vasculopathy in the early stages of pulmonary artery hypertension associated with systemic sclerosis.
abstract: BACKGROUND
  Pulmonary arterial hypertension (PAH) in patients with systemic sclerosis is associated with a poor prognosis, but this can be improved by early disease detection. Abnormal pulmonary and cardiac function can be detected early by means of echocardiography, whereas right heart catheterization is usually performed later.
  
  
  OBJECTIVE
  The purpose of this prospective study was to detect early the presence of pulmonary artery vasculopathy in patients with verified systemic sclerosis without significant pulmonary fibrosis, normal lung volumes and a mildly reduced lung diffusion capacity of carbon monoxide (DLCO).
  
  
  METHODS
  Nineteen consecutive female NYHA class I-II patients with scleroderma and a PAPs of < 35 mm/Hg measured by echocardiography, were enrolled between September 2007 and September 2009. They had a mean age of 51 +/- 13 years, body mass index of 25 +/- 5 kg/m2). They all underwent complete Doppler echocardiography, CPET, a pulmonary ventilation test (carbon monoxide lung diffusion, DLCO), HRCT. To investigate PAH by means of complete resting Doppler echocardiography estimates of systolic pulmonary artery pressure (PAPs) derived from tr icuspid regurgitation, mean PAP derived from pulmonary regurgitation, pulmonary vessel resistance (PVR) derived from the acceleration time of the pulmonary outflow tract (ACTpo), and right ventricular function derived from tricuspid annular plane systolic excursion (TAPSE). Right heart catheterisation was conducted only, if pulmonary hypertension was suggested by echocardiography and an abnormal ventilator test.The data are given as mean values +/- SD, unless otherwise stated. The correlations between the variables were analysed using Pearson's r coefficient, and the predictive value of the variables was calculated using linear regression analysis. A p value of > 0.05 was considered significant.
  
  
  RESULTS
  Right heart catheterization detected PAH in 15/19 patients; mean PAP was 30.5 mm/Hg and RVP 3.6 UW. Coronary angiography of the patients aged more than 55 years showed some evidence of significant coronary artery disease. Echocardiography showed high systolic PAP values (46 +/- 8 mmHg), whereas right ventricular function was normal (TAPSE 23 +/- 3 mm), and in line with the NYHA class. ACTpo was reduced in the patients with a systolic PAP of < 46 mm/Hg (p > 0.001) and positively correlated with DLCO (p > 0.001) and the hemodynamic data.There was a good correlation between ACTpo and PVR (hemodynamic data) (r = -0615; p > 0.01).
  
  
  CONCLUSIONS
  Although they need to be confirmed by studies of larger series of patients, our findings suggest that, in comparison with hemodynamic data, non-invasive echocardiographic measurements are an excellent means of identifying early-stage PAH.
authors.affiliation: Cardiopulmonary Dept, Cardiology Unit, University Hospital, Parma, Italy. wserra@libero.it
authors.forename: Walter
authors.initials: W
authors.lastname: Serra
authors.forename: Alfredo
authors.initials: A
authors.lastname: Chetta
authors.forename: Daniele
authors.initials: D
authors.lastname: Santilli
authors.forename: Flavio
authors.initials: F
authors.lastname: Mozzani
authors.forename: Pier Paolo
authors.initials: PP
authors.lastname: Dall'Aglio
authors.forename: Dario
authors.initials: D
authors.lastname: Olivieri
authors.forename: Maria Alberta
authors.initials: MA
authors.lastname: Cattabiani
authors.forename: Diego
authors.initials: D
authors.lastname: Ardissino
authors.forename: Tiziano
authors.initials: T
authors.lastname: Gherli
mesh_terms: D000328:Adult; D000368:Aged; D015150:Echocardiography, Doppler; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000000981:diagnostic imaging* / Q000503:physiopathology; D008875:Middle Aged; D011651:Pulmonary Artery / Q000000981:diagnostic imaging*; D012123:Pulmonary Ventilation; D012595:Scleroderma, Systemic / Q000150:complications*