Pulmonary Arterial Hypertension: From Diagnosis to Long-Term Management

Knowledge Track

Pulmonary Arterial Hypertension PAH remains one of the most clinically demanding conditions in cardiology: a rare, progressive disease where delayed diagnosis is common, risk stratification directly drives treatment choice, and the therapeutic landscape is shifting faster than most practice patterns can follow.

This Knowledge Track presents key evidence structured around the five clinical decisions that define PAH management today: confirming the diagnosis and classifying disease, applying risk stratification in practice, selecting the right upfront therapy, recognising treatment failure and escalating appropriately, and preparing for the novel mechanisms now entering clinical use, with each decision connecting evidence directly to clinical application.

Content selected and curated by ESC.

START HERE - Track Introduction

Before entering the structured learning path, these two resources establish the epidemiological and clinical context that underpins every module that follows: why PAH demands clinical attention, and why the gap between what is treatable and what is treated remains unacceptably wide.
The challenge in burden of pulmonary arterial hypertension: a perspective from the Global Burden of Disease Study
Congress Presentation
The challenge in burden of pulmonary arterial hypertension: a perspective from the Global Burden of Disease Study
29 August 2025 Core evidence: RV-PA pathophysiology and validated risk frameworks
Temporal trends in the incidence and mortality of pulmonary arterial hypertension in  the United Kingdom over the past 3 decades
Congress Presentation
Temporal trends in the incidence and mortality of pulmonary arterial hypertension in the United Kingdom over the past 3 decades
30 August 2025 Core evidence: RV-PA pathophysiology and validated risk frameworks

CLINICAL DECISION 01 - CONFIRMING PAH WITHIN THE PH SPECTRUM: How do I confirm PAH and avoid misclassifying it?

The diagnostic pathway for PAH requires more than a pressure measurement. This section covers the full clinical algorithm from first suspicion to haemodynamic confirmation, the practical implications of the updated mPAP threshold, and the most consequential differential in PAH practice: distinguishing Group 1 disease from postcapillary and HFpEF-driven phenotypes where PAH-specific vasodilators are unsafe.
The diagnostic approach to pulmonary hypertension
Congress Presentation
The diagnostic approach to pulmonary hypertension
30 August 2025 Core evidence: Foundational studies, risk frameworks, and guideline-level recommendations for PAH clinical practice
Patient phenotyping and pulmonary hypertension management: how to balance between different options
Congress Presentation
Patient phenotyping and pulmonary hypertension management: how to balance between different options
30 August 2025 Core evidence: Foundational studies, risk frameworks, and guideline-level recommendations for PAH clinical practice
Phenotyping of patients with pulmonary hypertension and mean pulmonary arterial pressure between 21 and 24 MMHG
Congress Presentation
Phenotyping of patients with pulmonary hypertension and mean pulmonary arterial pressure between 21 and 24 MMHG
30 August 2025 Core evidence: Foundational studies, risk frameworks, and guideline-level recommendations for PAH clinical practice
Phenotyping patients with precapillary pulmonary arterial hypertension PAH and a high probability of HFpEF
Congress Presentation
Phenotyping patients with precapillary pulmonary arterial hypertension PAH and a high probability of HFpEF
31 August 2025 Core evidence: Foundational studies, risk frameworks, and guideline-level recommendations for PAH clinical practice

CLINICAL DECISION 02 - RISK STRATIFICATION AND MONITORING: How do I assess severity, prognosis, and treatment response?

In PAH, risk stratification is not a one-time exercise — it is the mechanism that drives every treatment escalation decision. This module explains the RV-PA coupling concept that links vascular load to clinical outcomes, the validated risk models clinicians use in practice, and how echocardiographic parameters integrate with haemodynamic scores to guide real-world follow-up.
Pulmonary arterial hypertension and right ventricular-pulmonary arterial coupling
Congress Presentation
Pulmonary arterial hypertension and right ventricular-pulmonary arterial coupling
11 December 2025 Core evidence: Foundational studies, risk frameworks, and guideline-level recommendations for PAH clinical practice
Prediction models and scores in pulmonary hypertension
Congress Presentation
Prediction models and scores in pulmonary hypertension
1 September 2025 Core evidence: Foundational studies, risk frameworks, and guideline-level recommendations for PAH clinical practice
Mapping right ventricular adaptation in pulmonary arterial hypertension: insights from a six-phenotype model
Congress Presentation
Mapping right ventricular adaptation in pulmonary arterial hypertension: insights from a six-phenotype model
1 September 2025 Core evidence: Foundational studies, risk frameworks, and guideline-level recommendations for PAH clinical practice
Enhancing risk stratification in pulmonary hypertension: integrating echocardiographic parameters with the REVEAL Lite 2.0 score
Congress Presentation
Enhancing risk stratification in pulmonary hypertension: integrating echocardiographic parameters with the REVEAL Lite 2.0 score
30 August 2025 Core evidence: Foundational studies, risk frameworks, and guideline-level recommendations for PAH clinical practice
The added value of echocardiography in PAH risk assessment an AI machine learning-derived analysis of the ULTRA RIGHT VALUE registry
Congress Presentation
The added value of echocardiography in PAH risk assessment an AI machine learning-derived analysis of the ULTRA RIGHT VALUE registry
30 August 2025 Core evidence: Foundational studies, risk frameworks, and guideline-level recommendations for PAH clinical practice
Prognostic value of right ventricular myocardial work in patients with pulmonary arterial hypertension
Congress Presentation
Prognostic value of right ventricular myocardial work in patients with pulmonary arterial hypertension
13 December 2024 Core evidence: Foundational studies, risk frameworks, and guideline-level recommendations for PAH clinical practice
Improving risk prediction in pulmonary hypertension: the role of pulmonary arterial compliance
Congress Presentation
Improving risk prediction in pulmonary hypertension: the role of pulmonary arterial compliance
18 May 2025 Advanced reading: Specialist methodology, emerging trial data, and complex PAH management scenarios

CLINICAL DECISION 03 - PAH TREATMENT DECISIONS AND ESCALATION: Which treatment, when, and for whom?

Guideline-directed PAH therapy has moved firmly toward upfront combination and early escalation — but the decisions are rarely straightforward. This module covers the full treatment algorithm from initial strategy through oral and parenteral escalation, the ZENITH trial data on sotatercept that is reshaping high-risk management, and the evidence on treatment timing that every prescribing clinician needs to know.
Treatment strategies in pulmonary arterial hypertension: haemodynamics and proteomics
Congress Presentation
Treatment strategies in pulmonary arterial hypertension: haemodynamics and proteomics
30 August 2025 Core evidence: Foundational studies, risk frameworks, and guideline-level recommendations for PAH clinical practice
Safety and efficacy of macitentan and riociguat upfront combination therapy in patients with idiopathic pulmonary arterial hypertension at intermediate risk.
Congress Presentation
Safety and efficacy of macitentan and riociguat upfront combination therapy in patients with idiopathic pulmonary arterial hypertension at intermediate risk.
29 August 2025 Core evidence: Foundational studies, risk frameworks, and guideline-level recommendations for PAH clinical practice
Comparison of triple oral and triple parenteral combination therapy in pulmonary arterial hypertension
Congress Presentation
Comparison of triple oral and triple parenteral combination therapy in pulmonary arterial hypertension
30 August 2025 Core evidence: Foundational studies, risk frameworks, and guideline-level recommendations for PAH clinical practice
Hemodynamic effects of add-on therapies in pulmonary arterial hypertension: an additive component network meta-analysis.
Congress Presentation
Hemodynamic effects of add-on therapies in pulmonary arterial hypertension: an additive component network meta-analysis.
30 August 2025 Core evidence: Foundational studies, risk frameworks, and guideline-level recommendations for PAH clinical practice
Sotatercept Improves Hemodynamics and Right Heart Function in High-Risk Pulmonary Arterial Hypertension (PAH): Results from ZENITH Trial
Congress Presentation
Sotatercept Improves Hemodynamics and Right Heart Function in High-Risk Pulmonary Arterial Hypertension (PAH): Results from ZENITH Trial
30 August 2025 Core evidence: Foundational studies, risk frameworks, and guideline-level recommendations for PAH clinical practice
Dramatic differences resulting from treatment timing of treprostinil in high-risk patients: a real-world data analysis
Congress Presentation
Dramatic differences resulting from treatment timing of treprostinil in high-risk patients: a real-world data analysis
30 August 2025 Core evidence: Foundational studies, risk frameworks, and guideline-level recommendations for PAH clinical practice
Acutely decompensated arterial pulmonary hypertension: the hardest nut to crack? - discussion
Congress Presentation
Acutely decompensated arterial pulmonary hypertension: the hardest nut to crack? - discussion
21 March 2026 Acutely decompensated arterial pulmonary hypertension: the hardest nut to crack? - discussion

CLINICAL DECISION 04 - PAH IN COMPLEX POPULATIONS: How does PAH management change in high-risk subgroups?

Three patient groups demand a fundamentally different clinical approach: pregnant women, where PAH carries historically high maternal mortality; children, where trial evidence is almost entirely absent; and patients with connective tissue disease, where SSc-associated PAH carries the worst prognosis among all PAH subtypes.
2025 ESC Guidelines for the Management of Cardiovascular Disease and Pregnancy
Congress Presentation
2025 ESC Guidelines for the Management of Cardiovascular Disease and Pregnancy
29 August 2025 Core evidence: Foundational studies, risk frameworks, and guideline-level recommendations for PAH clinical practice
Pediatric pulmonary arterial hypertension: characteristics and efficacy of specific therapy on clinical, echocardiographic and hemodynamic parameters
Congress Presentation
Pediatric pulmonary arterial hypertension: characteristics and efficacy of specific therapy on clinical, echocardiographic and hemodynamic parameters
30 August 2025 Core evidence: Foundational studies, risk frameworks, and guideline-level recommendations for PAH clinical practice
Pulmonary arterial hypertension and connective tissue disease "multidisciplinar management, beyond a utopia"
Congress Presentation
Pulmonary arterial hypertension and connective tissue disease "multidisciplinar management, beyond a utopia"
18 May 2025 Core evidence: Foundational studies, risk frameworks, and guideline-level recommendations for PAH clinical practice

CLINICAL DECISION 05 - FUTURE OF PAH: PRECISION AND REVERSE REMODELLING: Where is PAH heading and how do I stay current?

PAH treatment is undergoing a conceptual shift — from managing symptoms and slowing progression to targeting vascular remodelling reversal. This module presents the precision medicine framework that is reshaping how patients are classified and matched to therapy, and the TKI pipeline that represents the next generation of disease-modifying treatment beyond sotatercept.
Precision medicine in pulmonary arterial hypertension: the right time?
Congress Presentation
Precision medicine in pulmonary arterial hypertension: the right time?
30 August 2025 Core evidence: Foundational studies, risk frameworks, and guideline-level recommendations for PAH clinical practice
Tyrosine kinase inhibitors
Congress Presentation
Tyrosine kinase inhibitors
30 August 2025 Core evidence: Foundational studies, risk frameworks, and guideline-level recommendations for PAH clinical practice