Haemodynamic definitions and updated clinical classification of pulmonary hypertension

Gérald Simonneau, David Montani, David S Celermajer, Christopher P Denton, Michael A Gatzoulis, Michael Krowka, Paul G Williams, Rogerio Souza, Gérald Simonneau, David Montani, David S Celermajer, Christopher P Denton, Michael A Gatzoulis, Michael Krowka, Paul G Williams, Rogerio Souza

Abstract

Since the 1st World Symposium on Pulmonary Hypertension (WSPH) in 1973, pulmonary hypertension (PH) has been arbitrarily defined as mean pulmonary arterial pressure (mPAP) ≥25 mmHg at rest, measured by right heart catheterisation. Recent data from normal subjects has shown that normal mPAP was 14.0±3.3 mmHg. Two standard deviations above this mean value would suggest mPAP >20 mmHg as above the upper limit of normal (above the 97.5th percentile). This definition is no longer arbitrary, but based on a scientific approach. However, this abnormal elevation of mPAP is not sufficient to define pulmonary vascular disease as it can be due to an increase in cardiac output or pulmonary arterial wedge pressure. Thus, this 6th WSPH Task Force proposes to include pulmonary vascular resistance ≥3 Wood Units in the definition of all forms of pre-capillary PH associated with mPAP >20 mmHg. Prospective trials are required to determine whether this PH population might benefit from specific management.Regarding clinical classification, the main Task Force changes were the inclusion in group 1 of a subgroup "pulmonary arterial hypertension (PAH) long-term responders to calcium channel blockers", due to the specific prognostic and management of these patients, and a subgroup "PAH with overt features of venous/capillaries (pulmonary veno-occlusive disease/pulmonary capillary haemangiomatosis) involvement", due to evidence suggesting a continuum between arterial, capillary and vein involvement in PAH.

Conflict of interest statement

Conflict of interest: G. Simonneau reports grants, personal fees and non-financial support from Actelion Pharmaceuticals, Bayer Healthcare, Merck and GSK, outside the submitted work. Conflict of interest: D. Montani reports grants, personal fees and non-financial support from Actelion Pharmaceuticals, Bayer Healthcare, Merck and GSK, outside the submitted work. Conflict of interest: D.S. Celermajer is an investigator on two clinical trials sponsored by Actelion. Conflict of interest: C.P. Denton reports grants and personal fees from Actelion and Roche, grants from GSK, and personal fees from Bayer and Boehringer Ingelheim, during the conduct of the study; grants and personal fees from Inventiva and CSL Behring, and personal fees from Leadiant, outside the submitted work. Conflict of interest: M.A. Gatzoulis reports personal fees for steering committee membership from Actelion Pharmaceuticals, and grants from Actelion Global, Pfizer and GSK, during the conduct of the study. Conflict of interest: M. Krowka is a steering committee member for PORTICO (Macitentan for Portopulmonary Hypertension Study), which is sponsored by Actelion, outside the submitted work. Conflict of interest: P.G. Williams received personal fees for advisory board meetings from Aspen SA and GSK, outside the submitted work. Conflict of interest: R. Souza reports lecture and consultancy fees from Actelion, Bayer, GSK and Pfizer, outside the submitted work.

Copyright ©ERS 2019.

Figures

FIGURE 1
FIGURE 1
The slope of the mean pulmonary arterial pressure (mPAP)–cardiac output (CO) relationship is different in normal control versus pulmonary arterial hypertension (PAH) subjects. Reproduced and modified from [26] with permission.
FIGURE 2
FIGURE 2
Knowledge of the mean pulmonary arterial pressure (mPAP)–cardiac output (CO) relationship does not allow distinction between left heart disease (LHD) and pulmonary vascular disease (PVD) patients; knowledge of exercise pulmonary arterial wedge pressure is also required. TPR: total pulmonary resistance; WU: Wood Units. Reproduced from [29] with permission.

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