A multicentric quality-control study of exercise Doppler echocardiography of the right heart and the pulmonary circulation. The RIGHT Heart International NETwork (RIGHT-NET)

Purpose This study was a quality-control study of resting and exercise Doppler echocardiography (EDE) variables measured by 19 echocardiography laboratories with proven experience participating in the RIGHT Heart International NETwork. Methods All participating investigators reported the requested variables from ten randomly selected exercise stress tests. Intraclass correlation coefficients (ICC) were calculated to evaluate the inter-observer agreement with the core laboratory. Inter-observer variability of resting and peak exercise tricuspid regurgitation velocity (TRV), right ventricular outflow tract acceleration time (RVOT Act), tricuspid annular plane systolic excursion (TAPSE), tissue Doppler tricuspid lateral annular systolic velocity (S’), right ventricular fractional area change (RV FAC), left ventricular outflow tract velocity time integral (LVOT VTI), mitral inflow pulsed wave Doppler velocity (E), diastolic mitral annular velocity by TDI (e’) and left ventricular ejection fraction (LVEF) were measured. Results The accuracy of 19 investigators for all variables ranged from 99.7 to 100%. ICC was > 0.90 for all observers. Inter-observer variability for resting and exercise variables was for TRV = 3.8 to 2.4%, E = 5.7 to 8.3%, e’ = 6 to 6.5%, RVOT Act = 9.7 to 12, LVOT VTI = 7.4 to 9.6%, S’ = 2.9 to 2.9% and TAPSE = 5.3 to 8%. Moderate inter-observer variability was found for resting and peak exercise RV FAC (15 to 16%). LVEF revealed lower resting and peak exercise variability of 7.6 and 9%. Conclusions When performed in expert centers EDE is a reproducible tool for the assessment of the right heart and the pulmonary circulation.

P1790 Is there any role of exercise Doppler echocardiography to unmask pulmonary hypertension (PH) in patients with systemic sclerosis and baseline echocardiographic measurements for PH in the gray zone?

Background The usefulness of exercise Doppler echocardiography (EDE) is uncertain in identifying patients with pulmonary hypertension (PH). Recently the cut off value for the definition of PH was changed and was set at the level of mean pulmonary arterial pressure (PAP) >20 mmHg, measured by right heart catheterization (RHC). Purpose We examined whether EDE can unmask the presence of PH in patients with systemic sclerosis (SSc) whose baseline echocardiographic assessment for PH is non-diagnostic. Methods Forty-one patients with SSc (2 men and 39 women; mean age 61.2 ± 10 years) underwent treadmill symptom-limited EDE using a modified Bruce protocol. Tricuspid regurgitation velocity (TRV) was recorded within 60 seconds after the termination of the test. Inclusion criteria comprised: preserved left (ejection fraction >50%), and right ventricular function (tricuspid annulus plane systolic excursion >15 mm), lack of left side moderate or severe valvulopathy, presence of mild or moderate TR, satisfactory exercise tolerance and baseline TRV in the range of 2.6-3.0 m/s. All patients had RHC within 48 hours after EDE. Results In 5 cases the quality of post-exercise TRV was poor and further analysis was confined to 36 patients. RHC confirmed the presence of PH (mean PAP >20 mmHg) in 14 cases (38.9%). All patients had pulmonary capillary wedge pressure <15 mmHg. The increase in TRV from baseline to post-exercise was 1.25 ± 0.6 m/s. Ten patients developed post-exercise TRV ≥3.7 m/s of whom in 8 RHC validated PH. Post-exercise TRV was positively correlated with mean PAP obtained by RHC (r = 0,652, p < 0.001). A cut-off value of post-exercise TRV ≥3.7 m/s had a sensitivity of 57.1%, a specificity of 90% and a diagnostic accuracy of 77.8% in detecting PH validated by RHC. Conclusions EDE has a moderate diagnostic accuracy for the identification of PH in patients with SSc whose baseline echocardiographic measurements for PH lie in the gray zone.

P6478Resting and exercise pulmonary artery systolic pressure to rule out later development of pulmonary arterial hypertension in systemic sclerosis

Background Patients with systemic sclerosis (SSc) are at risk of developing pulmonary arterial hypertension (PAH), which is often diagnosed late when the benefits of vasoactive therapies are limited. The concept of exercise pulmonary hypertension as a possible transitional phase anticipating resting PAH has been assessed in several pathologies, but has not been endorsed by the latest European Guidelines, because not supported by sufficient data. Purpose To evaluate whether PASP values at rest and at peak exercise, estimated at echocardiography, could be predictors of further development of PAH. Methods Four hundred and twenty-nine SSc patients without a previous diagnosis of PAH, enrolled at 4 referral Centres, underwent standard exercise Doppler echocardiography with PASP estimation at rest and at peak stress. Patients were then followed-up to assess the development of PAH, as diagnosed by a complete diagnostic work-up including right heart catheterization. PAH was defined by pre-capillary pulmonary hypertension (mean pulmonary artery pressure ≥25 mmHg with pulmonary arterial wedge pressure ≤15 mmHg), without significant interstitial lung disease and/or left heart disease. Results During the median follow-up of 75 months (IQR 29–114), 16 patients developed PAH. A combined cut-off of ≥24 mmHg as resting PASP and ≥40 as peak PASP was identified as the best predictor of further development of PAH (see Figure). Both resting PASP and peak PASP were predictors of PAH at univariate analysis (resting PASP OR 1.13, 95% C.I. 1.07–1.19, p<0.0001; peak PASP OR 1.13, 95% C.I. 1.07–1.18, p<0.0001). At multivariate analysis, only peak PASP was independently associated to PAH development (OR 1.13, 95% C.I. 1.04–1.18, p<0.001). Only one patient among those with resting PASP <24 mmHg and peak PASP <40 mmHg (34.7% of the total population) developed PAH during the follow-up (after 10 years from normal exercise Doppler echocardiography). Kaplan-Meier curves Conclusions Exercise increase in PASP is an independent predictor of later development of PAH in SSc. An increase in exercise PASP is frequent and is not necessarily associated with a later development of PAH, whereas the very high negative predictive value of a normal PASP both at rest and at peak exercise can be used in the clinical practice to confidently rule out about one third of patients. Acknowledgement/Funding Italian Ministry of Health (Ricerca Finalizzata 2011-2012)