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In patients with pulmonary hypertension (PH) right ventricular (RV) function is a well-recognized determinant of clinical outcome, including cardiac death. Data regarding the prognostic value of comorbidities in patients with PH are scarce. Purpose. To identify predictors of cardiac death and all-cause mortality on mid-term follow-up in PH patients receiving specific vasodilator therapy. Methods. Sixty-eight patients (40 ± 16 years, 51 women) with PH (12 with idiopathic pulmonary arterial hypertension-PAH, 32 with congenital heart disease, 10 ith connective tissue disease, 7 with chronic thromboembolic PH and 7 with other forms of PAH) treated with pulmonary vasodilators were studied. Clinical parameters (NYHA class, 6-minutes walking distance, 6MWD), biological (hemoglobin, B-type natriuretic peptide-BNP), and echocardiographic parameters of RV function (TAPSE, tissue-Doppler imaging-derived free-RV wall S- and e’-wave, RV fractional area change (FAC), Tei index, I/H, RV isovolumic acceleration-IVA) and cardiac index were assessed. RV global longitudinal strain (RV-GLS) was measured from the apical 4-chamber view as the average of 3 (free-wall) and 6 (free-wall and interventricular septum) segments by 2D speckle-tracking echocardiography. Parameters of pulmonary artery stiffness (PAS) were assessed: pulsatility, capacitance, elastic modulus, dynamic compliance. Two endpoints were defined: cardiac death and all-cause mortality. Results. PH patients were followed-up for 35 months (4-81). Comorbidities were identified in 15 patients (non-cardiac surgery, anemia, chronic kidney disease, neoplasia, respiratory infections). During follow-up, 20 patients died, 14 deaths being cardiac deaths. At univariable analysis, the following parameters correlated with all-cause mortality: age (51 ± 14 in deceased patients vs 36 ± 15 years in surviving patients, p &amp;lt; 0.001), 6MWD (262 ± 171 vs 369 ± 155 m, p = 0.015), FAC (30 ± 9 vs 38 ± 8%, p &amp;lt; 0.001), 6 segments RV-GLS (-12.3 ± 5.1 vs -15.1 ± 4.5%, p = 0.025), S-RV (9.9 ± 2.2 vs 11.4 ±2.3 cm/s, p = 0.016), TAPSE (16.3 ± 3.3 vs 18.6 ± 3.5 mm, p = 0.014), right atrial (RA) area (30.6 ± 13.1 vs 21.2 ± 8.8 cm2, p = 0.001) hemoglobin (12.9 ± 2.6 vs 15.2 ± 3.0 g/dl, p = 0.004) and BNP levels (lnBNP, 5.8 ± 1.3 vs 4.1 ± 1.4, p &amp;lt; 0.001), presence of comorbidities (10 vs 5, p = 0.001). At multivariable analysis, only comorbidities (p = 0.05) and FAC (p = 0.07) seemed to independently predict all-cause mortality. Predictors of cardiac death at univariate analysis were age, RV function parameters, RA area, PA capacitance (1.02 ± 0.56 vs 1.48 ± 0.80 ml/mmHg, p = 0.05) and cardiac index (2.48 ± 0.64 vs 3.31 ± 1.34 l/min/m2, p = 0.03) but only age (p = 0.03) and cardiac index (0.06) emerged as independent predictors of cardiac death. Conclusions: In PH patients, RV function and comorbidities predict all-cause mortality, while age and cardiac index are independent determinants of cardiac death. Identifying and properly treating associated pathologies could impact survival in this setting.

P1545 Predictors of survival in patients with pulmonary hypertension receiving specific vasodilator therapy