Improvement in Right Ventricular Strain with Ambrisentan and Tadalafil Upfront Therapy in Scleroderma-associated Pulmonary Arterial Hypertension

ARTICLE: Improvement in Right Ventricular Strain with Ambrisentan and Tadalafil Upfront Therapy in Scleroderma-associated Pulmonary Arterial Hypertension

AUTHORS: Valentina MercurioMonica MukherjeeRyan J. Tedford, Roham T. Zamanian, Rubina M. Khair, Takahiro Sato, Omar A. Minai, Fernando Torres, Reda E. Girgis, Kelly Chin, Rachel DamicoTodd M. Kolb, Stephen C. Mathai and Paul M. Hassoun

JOURNAL: Am J Respir Crit Care Med. 2018 Feb 1;197(3):388-391. doi: 10.1164/rccm.201704-0789LE.

To the Editor:

Scleroderma-associated pulmonary arterial hypertension (SSc-PAH) is characterized by poor survival, mainly related to the development of right ventricular (RV) failure (1). Although recent advances in PAH-specific treatment have improved survival, patients with SSc-PAH have a modest response to therapy and higher mortality compared with those with PAH due to other etiologies (12). Initial upfront PAH combination therapy is a novel approach aimed at improving outcomes (3).

In a recent prospective, multicenter, open-label study of treatment-naive patients with SSc-PAH (the ATPAHSS [Ambrisentan and Tadalafil in Pulmonary Arterial Hypertension Associated with Systemic Sclerosis] trial; NCT01042158), initial upfront therapy with a phosphodiesterase-5 inhibitor (tadalafil 40 mg oral once daily) and an endothelin receptor antagonist (ambrisentan 10 mg oral once daily) demonstrated improvement in invasive hemodynamics and functional status (4). Because survival is predominantly dependent on RV function (5), we evaluated the effects of such treatment on RV morphology and function using conventional and novel speckle tracking–derived echocardiography in the ATPAHSS trial cohort. Some of the results of this study have been previously reported in abstract form (6).

Full details regarding the methods used are provided in Reference 4 and its online supplement. Two-dimensional and M-mode echocardiography was performed locally at each site according to the American Society of Echocardiography guidelines (78). The results were stored on CDs and sent to the core laboratory at Johns Hopkins University (Baltimore, MD).

Two echocardiographers (V.M. and M.M.) blinded to patient information, clinical variables, and timing of study acquisition performed echocardiographic analysis offline using Synapse cardiovascular software (V4.0.8; Fujifilm Medical Systems U.S.A., Inc.) for the conventional analysis, and a commercially available vendor-independent strain software (Epsilon) for the RV longitudinal systolic strain (RVLSS) analysis.

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