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. 2024 Sep 5;64(3):2400197.
doi: 10.1183/13993003.00197-2024. Print 2024 Sep.

Risk stratification refinements with inclusion of haemodynamic variables at follow-up in patients with pulmonary arterial hypertension

Athénaïs Boucly  1   2   3   4 , Antoine Beurnier  1   3   5 , Ségolène Turquier  6 , Mitja Jevnikar  1   2   3 , Pascal de Groote  7 , Ari Chaouat  8 , Céline Cheron  1   2   3 , Xavier Jaïs  1   2   3 , François Picard  9 , Grégoire Prévot  10 , Anne Roche  1   2   3 , Sabina Solinas  1   2   3 , Vincent Cottin  6 , Fabrice Bauer  11 , David Montani  1   2   3 , Marc Humbert  1   2   3 , Laurent Savale  1   2   3   12 , Olivier Sitbon  13   2   3   12 ; PulmoTension Network
Affiliations

Risk stratification refinements with inclusion of haemodynamic variables at follow-up in patients with pulmonary arterial hypertension

Athénaïs Boucly et al. Eur Respir J. .

Abstract

Background: Haemodynamic variables are prognostic factors in pulmonary arterial hypertension (PAH). However, right heart catheterisation (RHC) is not systematically recommended to assess the risk status during follow-up. This study aimed to assess the added value of haemodynamic variables in prevalent patients to predict the risk of death or lung transplantation according to their risk status assessed by the non-invasive four-strata model as recommended by the European guidelines.

Methods: We evaluated incident patients with PAH enrolled in the French pulmonary hypertension registry between 2009 and 2020 who had a first follow-up RHC. Cox regression identified, in each follow-up risk status, haemodynamic variables significantly associated with transplant-free survival. Optimal thresholds were determined by time-dependent receiver operating characteristics. Several multivariable Cox regression models were performed to identify the haemodynamic variables improving the non-invasive risk stratification model.

Results: We analysed 1240 incident patients reassessed within 1 year by RHC. None of the haemodynamic variables were significantly associated with transplant-free survival among low-risk (n=386) or high-risk (n=71) patients. Among patients at intermediate (intermediate-low, n=483 and intermediate-high, n=300) risk at first follow-up, multivariable models including either stroke volume index (SVI) or mixed venous oxygen saturation (S vO2 ) were the best. The prognostic performance of a refined six-strata risk stratification model including the non-invasive four-strata model and SVI >37 mL·m-2 and/or S vO2 >65% for patients at intermediate risk (area under the curve (AUC) 0.81; c-index 0.74) was better than that of the four-strata model (AUC 0.79, p=0.009; c-index 0.72).

Conclusion: Cardiopulmonary haemodynamics may improve risk stratification at follow-up in patients at intermediate risk.

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Conflict of interest statement

Conflict of interest: A. Boucly reports grants from Acceleron, Janssen and MSD, and lecture honoraria and travel support from Janssen, Merck, AOP Orphan and Ferrer, outside the submitted work. S. Turquier reports consulting fees from Ferrer, lecture honoraria from MSD, and travel support from Ferrer, MSD and Janssen, outside the submitted work. P. de Groote reports consulting fees from Boehringer Ingelheim, Servier, Bayer and MSD, and lecture honoraria from BMS, Novartis, Vifor and Pfizer, outside the submitted work. A. Chaouat reports travel support from MSD and Janssen, outside the submitted work. C. Cheron reports travel support from MSD, outside the submitted work. X. Jaïs reports grants from Acceleron, Janssen, MSD and Bayer HealthCare, and lecture honoraria from Janssen and MSD, outside the submitted work. F. Picard reports lecture honoraria from Bayer, Boehringer Ingelheim/Lilly, Novartis, MSD, AstraZeneca and Novo Nordisk, and travel support from Bayer and Novartis, outside the submitted work. G. Prévot reports lecture honoraria and travel support from Janssen and MSD, outside the submitted work. V. Cottin reports consulting fees from Ferrer/United Therapeutics and Liquidia, and lecture honoraria from Ferrer/United Therapeutics, outside the submitted work. D. Montani reports grants from Acceleron, Merck MSD and Janssen, consulting fees from Acceleron, Merck MSD, Janssen and Ferrer; and lecture honoraria from Bayer, Janssen, Boehringer, Chiesi, GSK, Ferrer and Merck MSD, outside the submitted work. M. Humbert reports grants from Acceleron, AOP Orphan, Janssen, Merck and Shou Ti, consulting fees from 35 Pharma, Aerovate, AOP Orphan, Bayer, Chiesi, Ferrer, Janssen, Keros, Merck, MorphogenIX, Shou Ti and United Therapeutics, lecture honoraria from Janssen and Merck, and advisory board participation with Acceleron, Altavant, Janssen, Merck and United Therapeutics, outside the submitted work. L. Savale reports lecture honoraria and travel support from MSD and Janssen and Janssen, outside the submitted work. O. Sitbon reports grants from Aerovate, AOP Orphan, Ferrer, Janssen and MSD, consulting fees from Altavant/Enzyvant, AOP Orphan, Ferrer, Gossamer Bio, Janssen, Liquidia, MSD and Respira Therapeutics, lecture honoraria from AOP Orphan, Janssen, Ferrer and MSD, and advisory board participation with Altavant/Enzyvant, Gossamer Bio and Janssen, outside the submitted work. The remaining authors have no potential conflicts of interest to disclose.

Figures

None
1240 pulmonary arterial hypertension (PAH) patients from the French pulmonary hypertension (PH) registry reassessed at first follow-up visit by three non-invasive variables (World Health Organization/New York Heart Association Functional Class (WHO/NYHA FC), 6-min walk distance (6MWD) and brain natriuretic peptide (BNP)/N-terminal pro-BNP) and right heart catheterisation. 1- and 3-year transplant-free survival rates. Cardiopulmonary haemodynamics improve risk stratification at follow-up in patients at intermediate risk. SVI: stroke volume index; SvO2: mixed venous oxygen saturation.
FIGURE 1
FIGURE 1
Patient flowchart. PAH: pulmonary arterial hypertension; PVOD: pulmonary veno-occlusive disease; CHD: congenital heart disease; POPH: portopulmonary hypertension; WHO/NYHA FC: World Health Organization/New York Heart Association Functional Class; 6MWD: 6-min walk distance; BNP: brain natriuretic peptide; NT-proBNP: N-terminal pro-BNP; RHC: right heart catheterisation.
FIGURE 2
FIGURE 2
Kaplan–Meier survival curves in each at first follow-up risk status according to haemodynamic variables in 1019 pulmonary arterial hypertension patients with at least stroke volume index (SVI) or mixed venous oxygen saturation (SvO2) available at first follow-up reassessment: a) low risk, b) intermediate-low risk, c) intermediate-high risk and d) high risk. A good haemodynamic profile (Good HD) was defined by at least one criterion among SVI >37 mL·m−2 and SvO2 >65%. A poor haemodynamic profile (Poor HD) was defined as neither SVI >37 mL·m−2 nor SvO2 >65%.
FIGURE 3
FIGURE 3
Refined algorithm based on the non-invasive four-strata model and right heart catheterisation variables developed in 1019 pulmonary arterial hypertension patients with at least stroke volume index (SVI) or mixed venous oxygen saturation (SvO2) available at first follow-up reassessment. 1- and 3-year transplant-free survival rates.
FIGURE 4
FIGURE 4
Kaplan–Meier survival curves according to risk status calculated from the three non-invasive variables (World Health Organization/New York Heart Association Functional Class, 6-min walk distance and brain natriuretic peptide (BNP)/N-terminal pro-BNP) and stroke volume index (SVI) and/or mixed venous oxygen saturation (SvO2).
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