Echocardiography may help detect pulmonary vasculopathy in the early stages of pulmonary artery hypertension associated with systemic sclerosis

Abstract

BackgroundPulmonary 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.ObjectivesThe 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).MethodsNineteen 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.ResultsRight 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).ConclusionsAlthough 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.

Assessment of the relationship between right ventricular function and the severity of obstructive sleep‐disordered breathing

“…RV outflow acceleration time (AcT) and tricuspid regurgitation velocity were measured to determine if there was any evidence of pulmonary hypertension. Results were considered suggestive of pulmonary hypertension when the tricuspid regurgitation velocity was >2.8 m/s without pulmonary valve stenosis , when pulmonary artery (PA) AcT was <90 ms, or when PA AcT/PA ejection time (ET) was <0.3 . All measurements were made at end expiration.…”

Risk factors associated with pulmonary arterial hypertension in patients with systemic sclerosis and implications for screening

“…Combining echocardiography-derived Ppa,syst o35 mmHg with right ventricular Tei-index .0.36 was shown to improve the accuracy of PAH-SSc detection as confirmed by right heart catheterisation from 80% to 90% [42]. Evaluation of echocardiography-derived Ppa, post-systolic tricuspid annular movement (TAPSE) as a marker of right ventricular function, and the acceleration time of right outflow as an indirect marker of PVR suggested early-stage PAH in 19 patients with normal lung volumes, no significant pulmonary fibrsois and mildly reduced DL,CO (mean¡SD 71¡18% of theoretical), which was subsequently confirmed by right heart catheterisation in 15 (79%) patients [43].…”

Potential benefit of bosentan therapy in borderline or less severe pulmonary hypertension secondary to idiopathic pulmonary fibrosis—an interim analysis of results from a prospective, single-center, randomized, parallel-group study

“…ECG examinations were carried out during the run-in period* and every 6 months thereafter**. Complete two-dimensional, pulsed-wave, color-flow echocardiography was performed using the Toshiba ultrasound system Xario (TOSHIBA MEDICAL SYSTEMS CORPORATION, Tochigi, Japan) as previously described [ 14 – 25 ]. (See also Additional file 3 ).…”