• Myriam Amsallem, Andrew J Sweatt, Marie C Aymami, Tatiana Kuznetsova, Mona Selej, HongQuan Lu, Olaf Mercier, Elie Fadel, Ingela Schnittger, Michael V McConnell, Marlene Rabinovitch, Roham T Zamanian, and Francois Haddad.
• From the Division of Cardiovascular Medicine (M.A., M.C.A., M.S., H.L., I.S., M.V.M., F.H.), Cardiovascular Institute (M.A., M.C.A., H.L., I.S., M.V.M., F.H.), Division of Pulmonary and Critical Care Medicine (A.J.S., R.T.Z.), Vera Moulton Wall Center at Stanford (M.R., R.T.Z., F.H.), and Division of Pediatrics (M.R.), Stanford University School of Medicine, CA; Research Unit Hypertension and Cardiovascular Epidemiology, KU Leuven Department of Cardiovascular Sciences, University of Leuven, Belgium (T.K.); and Division of Cardiothoracic Surgery, Marie Lannelongue Hospital, Le Plessis Robinson, France (O.M., E.F., M.A.). mamsallem@stanford.edu.
• Circ Cardiovasc Imaging. 2017 Jun 1; 10 (6).

BackgroundRight ventricular (RV) end-systolic dimensions provide information on both size and function. We investigated whether an internally scaled index of end-systolic dimension is incremental to well-validated prognostic scores in pulmonary arterial hypertension.Methods And ResultsFrom 2005 to 2014, 228 patients with pulmonary arterial hypertension were prospectively enrolled. RV end-systolic remodeling index (RVESRI) was defined by lateral length divided by septal height. The incremental values of RV free wall longitudinal strain and RVESRI to risk scores were determined. Mean age was 49±14 years, 78% were female, 33% had connective tissue disease, 52% were in New York Heart Association class ≥III, and mean pulmonary vascular resistance was 11.2±6.4 WU. RVESRI and right atrial area were strongly connected to the other right heart metrics. Three zones of adaptation (adapted, maladapted, and severely maladapted) were identified based on the RVESRI to RV systolic pressure relationship. During a mean follow-up of 3.9±2.4 years, the primary end point of death, transplant, or admission for heart failure was reached in 88 patients. RVESRI was incremental to risk prediction scores in pulmonary arterial hypertension, including the Registry to Evaluate Early and Long-Term PAH Disease Management score, the Pulmonary Hypertension Connection equation, and the Mayo Clinic model. Using multivariable analysis, New York Heart Association class III/IV, RVESRI, and log NT-proBNP (N-Terminal Pro-B-Type Natriuretic Peptide) were retained (χ2, 62.2; P<0.0001). Changes in RVESRI at 1 year (n=203) were predictive of outcome; patients initiated on prostanoid therapy showed the greatest improvement in RVESRI. Among right heart metrics, RVESRI demonstrated the best test-retest characteristics.ConclusionsRVESRI is a simple reproducible prognostic marker in patients with pulmonary arterial hypertension.© 2017 American Heart Association, Inc.

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