Jason Weatherald1,2,3,4,5, Athénaïs Boucly6,2,3, Denis Chemla6,2,7, Laurent Savale6,2,3, Mingkai Peng8,5, Mitja Jevnikar6,2,3, Xavier Jaïs6,2,3, Yu Taniguchi6,2,3, Caroline O'Connell6,2,3, Florence Parent6,2,3, Caroline Sattler6,2,3, Philippe Hervé6,2,3, Gérald Simonneau6,2,3, David Montani6,2,3, Marc Humbert6,2,3, Yochai Adir9, Olivier Sitbon1,2,3. 1. Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France (J.W., A.B., D.C., L.S., M.J., X.J., Y.T., C.O., F.P., C.S., P.H., G.S., D.M., M.H., O.S.) olivier.sitbon@aphp.fr jcweathe@ucalgary.ca. 2. Service de Pneumologie (J.W., A.B., D.C., L.S., M.J., X.J., Y.T., C.O., F.P., C.S., P.H., G.S., D.M., M.H., O.S.). 3. INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France (J.W., A.B., L.S., M.J., X.J., Y.T., C.O., F.P., C.S., P.H., G.S., D.M., M.H., O.S.). 4. Department of Medicine, Division of Respirology (J.W.). 5. Libin Cardiovascular Institute of Alberta (J.W., M.P.), University of Calgary, Calgary, Canada. 6. Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France (J.W., A.B., D.C., L.S., M.J., X.J., Y.T., C.O., F.P., C.S., P.H., G.S., D.M., M.H., O.S.). 7. Service de Physiologie (D.C.), Hôpital Bicêtre, AP-HP, Le Kremlin-Bicêtre, France. 8. Department of Community Health Sciences (M.P.). 9. Lady Davis Carmel Medical Center, Pulmonary Division, Faculty of Medicine, Technion Institute of Technology, Haifa, Israel (Y.A.).
Abstract
BACKGROUND: Hemodynamic variables such as cardiac index and right atrial pressure have consistently been associated with survival in pulmonary arterial hypertension (PAH) at the time of diagnosis. Recent studies have suggested that pulmonary arterial compliance may also predict prognosis in PAH. The prognostic importance of hemodynamic values achieved after treatment initiation is less well established. METHODS: Our objective was to evaluate the prognostic importance of clinical and hemodynamic variables during follow-up, including pulmonary arterial compliance, after initial management in PAH. We evaluated incident patients with idiopathic, drug- and toxin-induced, or heritable PAH enrolled in the French pulmonary hypertension registry between 2006 and 2016 who had a follow-up right-sided heart catheterization (RHC). The primary outcome was death or lung transplantation. We used stepwise Cox regression and the Kaplan-Meier method to assess variables obtained at baseline and at first follow-up RHC. RESULTS: Of 981 patients, a primary outcome occurred in 331 patients (33.7%) over a median follow-up duration of 2.8 years (interquartile range, 1.1-4.6 years). In a multivariable model considering only baseline variables, no hemodynamic variables independently predicted prognosis. Median time to first follow-up RHC was 4.6 months (interquartile range, 3.7-7.8 months). At first follow-up RHC (n=763), New York Heart Association functional class, 6-minute walk distance, stroke volume index (SVI), and right atrial pressure were independently associated with death or lung transplantation, adjusted for age, sex, and type of PAH. Pulmonary arterial compliance did not independently predict outcomes at baseline or during follow-up. The adjusted hazard ratio for SVI was 1.28 (95% confidence interval, 1.11-1.49; P<0.01) per 10-mL/m2 decrease and for right atrial pressure was 1.05 (95% confidence interval, 1.02-1.09; P<0.01) per 1-mm Hg increase. Among patients who had 2 (n=355) or 3 (n=193) low-risk prognostic features at follow-up, including a cardiac index ≥2.5 L·min-1·m-2, 6-minute walk distance >440 m, and New York Heart Association class I or II functional class, lower SVI was still associated with higher rates of death or lung transplantation (P<0.01). CONCLUSIONS: SVI and right atrial pressure were the hemodynamic variables that were independently associated with death or lung transplantation at first follow-up RHC after initial PAH treatment. These findings suggest that the SVI could be a more appropriate treatment target than cardiac index in PAH.
BACKGROUND: Hemodynamic variables such as cardiac index and right atrial pressure have consistently been associated with survival in pulmonary arterial hypertension (PAH) at the time of diagnosis. Recent studies have suggested that pulmonary arterial compliance may also predict prognosis in PAH. The prognostic importance of hemodynamic values achieved after treatment initiation is less well established. METHODS: Our objective was to evaluate the prognostic importance of clinical and hemodynamic variables during follow-up, including pulmonary arterial compliance, after initial management in PAH. We evaluated incident patients with idiopathic, drug- and toxin-induced, or heritable PAH enrolled in the French pulmonary hypertension registry between 2006 and 2016 who had a follow-up right-sided heart catheterization (RHC). The primary outcome was death or lung transplantation. We used stepwise Cox regression and the Kaplan-Meier method to assess variables obtained at baseline and at first follow-up RHC. RESULTS: Of 981 patients, a primary outcome occurred in 331 patients (33.7%) over a median follow-up duration of 2.8 years (interquartile range, 1.1-4.6 years). In a multivariable model considering only baseline variables, no hemodynamic variables independently predicted prognosis. Median time to first follow-up RHC was 4.6 months (interquartile range, 3.7-7.8 months). At first follow-up RHC (n=763), New York Heart Association functional class, 6-minute walk distance, stroke volume index (SVI), and right atrial pressure were independently associated with death or lung transplantation, adjusted for age, sex, and type of PAH. Pulmonary arterial compliance did not independently predict outcomes at baseline or during follow-up. The adjusted hazard ratio for SVI was 1.28 (95% confidence interval, 1.11-1.49; P<0.01) per 10-mL/m2 decrease and for right atrial pressure was 1.05 (95% confidence interval, 1.02-1.09; P<0.01) per 1-mm Hg increase. Among patients who had 2 (n=355) or 3 (n=193) low-risk prognostic features at follow-up, including a cardiac index ≥2.5 L·min-1·m-2, 6-minute walk distance >440 m, and New York Heart Association class I or II functional class, lower SVI was still associated with higher rates of death or lung transplantation (P<0.01). CONCLUSIONS: SVI and right atrial pressure were the hemodynamic variables that were independently associated with death or lung transplantation at first follow-up RHC after initial PAH treatment. These findings suggest that the SVI could be a more appropriate treatment target than cardiac index in PAH.
Authors: Lauren Rose; Kurt W Prins; Stephen L Archer; Marc Pritzker; E Kenneth Weir; Jeffrey R Misialek; Thenappan Thenappan Journal: J Heart Lung Transplant Date: 2018-09-14 Impact factor: 10.247
Authors: Marco Vicenzi; Sergio Caravita; Irene Rota; Rosa Casella; Gael Deboeck; Lorenzo Beretta; Andrea Lombi; Jean-Luc Vachiery Journal: PLoS One Date: 2022-05-19 Impact factor: 3.752
Authors: Raymond L Benza; Mark Doyle; David Lasorda; Kishan S Parikh; Priscilla Correa-Jaque; Nima Badie; Greg Ginn; Sophia Airhart; Veronica Franco; Manreet K Kanwar; Srinivas Murali; Amresh Raina; Rahul Agarwal; Sudarshan Rajagopal; Jason White; Robert Biederman Journal: Chest Date: 2019-06-29 Impact factor: 9.410
Authors: Denis Chemla; Emmanuelle Berthelot; Jason Weatherald; Edmund M T Lau; Laurent Savale; Antoine Beurnier; David Montani; Olivier Sitbon; Pierre Attal; David Boulate; Patrick Assayag; Marc Humbert; Philippe Hervé Journal: ERJ Open Res Date: 2021-05-31
Authors: Hilary M DuBrock; Rodrigo Cartin-Ceba; Richard N Channick; Steven M Kawut; Michael J Krowka Journal: Chest Date: 2020-08-13 Impact factor: 9.410