The 6th World Symposium on Pulmonary Hypertension: what’s old is new

Abstract

In February 2018, the 6th World Symposium on Pulmonary Hypertension (WSPH) brought together experts from various disciplines to review the most relevant clinical and scientific advances in the field of PH over the last 5 years. Based on careful review and discussions by members of the different task forces, major revisions were made on the hemodynamic definition for various forms of PH and new genes were added to the list of genetic markers associated with pulmonary arterial hypertension (PAH) and pulmonary veno-occlusive disease. In addition, the use of risk stratification tools was encouraged as a strategy to reduce one-year mortality risk in PAH patients through early implementation of PAH therapies. While members of the medical community are still debating some of the proposed changes, the new WSPH guidelines advocate early diagnosis and initiation of combination therapy to reduce mortality and improve quality of life in patients with PH.

The Role and Regulation of Pulmonary Artery Smooth Muscle Cells in Pulmonary Hypertension

“…Specific drug treatment of WHO group 1 PAH by targeting the nitric oxide, endothelin, and prostaglandin pathways has been the standard since 2003. Recently, based on different risk stratification, monotherapy or dual-combination therapies, including macitentan and sildenafil, riociguat and bosentan, selexipag and endothelin receptor antagonist (ERA) or phosphodiesterase inhibitor (PDE5i), or both, are recommended [ 14 , 15 ].…”

Current and emerging therapeutic approaches to pulmonary hypertension

“…The 2015 pulmonary hypertension guidelines of the European Society of Cardiology (ESC)/European Respiratory Society (ERS) and the 6th WSPH congress Task Force (Nice, 2018) revised the definition of PAH ( Condon et al, 2019 ; Galie et al, 2016 ; Galie et al, 2019 ). Consequently, PAH is now hemodynamically defined as a mean pulmonary arterial pressure (mPAP) ≥ 20 mmHg at rest as assessed by right heart catheterization (RHC) and a pulmonary vascular resistance ≥ 3 Wood Units in the definition of all forms of pre-capillary pulmonary hypertension (PH) ( Condon et al, 2019 ; Galie et al, 2016 ; Galie et al, 2019 ). Recent data showed that the normal mPAP is 14 ± 3 mmHg at rest with an upper limit of normal of approximately 20 mmHg.…”

Current Understanding of Circulating Biomarkers in Pulmonary Hypertension Due to Left Heart Disease

“…Among these, PH attributed to LV diastolic dysfunction, also referred to as PH associated with heart failure with preserved ejection fraction (PH-HFpEF), is the most common form, although the reported prevalence varies from 23 to 83% due to variable definitions and diagnostic methods used to date (5)(6)(7). In addition to the already existing ambiguity in definition of this challenging syndrome, the 6th World Symposium on PH (Nice, 2018) has recently redefined PH-LHD as consisting of a mean pulmonary artery pressure (mPAP) greater than 20 mm Hg (revised from at least 25 mm Hg) and a pulmonary artery wedge pressure (PAWP) greater than 15 mm Hg (Figure 1) (8)(9)(10)(11). Although this change reflects recent reports describing increased risk of disease progression in patients with a mild elevation in mPAP (21 to 24 mm Hg), this revision may lead to further confusion among clinicians when trying to diagnose PH in patients (9,12).…”

“…These features have been associated with mortality and cardiac hospitalizations in patients with CpcPH associated with HFpEF (CpcPH-HFpEF) (26,27). However, the new definition from the 6th World Symposium on PH only includes PVR of less than 3 Wood units for IpcPH and PVR of at least 3 Wood units for CpcPH (Figure 1) (9)(10)(11).…”