A18 U GOT THE LOOK: DRUGS, TOXINS, GENES AND RISK ASSESSMENT IN PULMONARY HYPERTENSION: Improved Right Ventricular Chamber Size And Contractility After Upfront Combination Therapy With Ambrisentan And Tadalfil In Scleroderma-Associated Pulmonary Arterial Hypertension

• Published in: American journal of respiratory and critical care medicine Vol. 195
• Main Authors: Mercurio, V, Mukherjee, M, Tedford, R, Zamanian, R T, Khair, R, Sato, T, Minai, O, Torres, F, Girgis, R E, Chin, K, Damico, R L, Kolb, T M, Mathai, S, Hassoun, P M
• Format: Journal Article
• Language: English
• Published: New York American Thoracic Society 01.01.2017
• Subjects: Combination therapy; Hypertension; Scleroderma
• ISSN: 1073-449X; 1535-4970

In a study of treatment naive SSc-PAH patients (ATPAHSS Trial), initial upfront therapy with a endothelin receptor antagonist (ambrisentan) and phosphodiesterase inhibitor (tadalafil) and demonstrated improvement in hemodynamics and functional status. Echocardiographic analysis included standard assessment of RV chamber size and function using conventional measures of tricuspid annular plane systolic excursion (TAPSE), fractional area change (FAC), and tissue Doppler derived S' velocity; and novel RV longitudinal systolic speckle tracking strain (RVLSS) of the basal, mid and apical RV free wall. BASELINE 36 WEEKS p value NORMAL VALUES Age, y 59 ± 10 Female sex, n (%) 21 (91.3) Race White/Black/Other, n 20/2/1 WHO functional class I/II/III/IV, n 0/8/15/0 1/12/10/0 <0.05 Right atrial area, cm2 22.6 ± 5.3 19.3 ± 4.6 0.001 < 181 Right ventricular basal diameter, mm 45.9 ± 4.3 42.4 ± 4.4 <0.0001 < 421 Right ventricular end-diastolic area, cm2 25.1 ± 4.6 22.4 ± 5.1 <0.01 < 251 Right ventricular end-systolic area, cm2 18.6 ± 5.1 14.1 ± 5.2 <0.0001 < 141 Fractional area change, % 26.6 ± 9.9 38.4 ± 7.9 <0.0001 > 351 TAPSE, mm 16.1 ± 3.5 22.5 ± 3.2 <0.0001 > 161 S' wave, cm/sec (N=10) 10.3 ± 2.2 10.7 ± 1.3 <0.05 > 101 Basal RVLLS, % -18.1 ± 8.2 -26.8 ± 9.7 <0.01 -25 ± 62 Midventricular RVLLS, % -13.5 ± 6.6 -18.8 ± 6.8 0.001 -27 ± 52 Apical RVLLS, % -10.1 ± 5.9 -11.6 ± 6.0 NS -24 ± 62 Global RVLLS, % -13.9 ± 5.2 -19.1 ± 5.4 0.001 -26 ± 42 This abstract is funded by: