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2022 ESC/ERS Pulmonary Hypertension Guidelines

Clinical Quick Reference — Diagnosis and Treatment

Published: European Heart Journal (2022)
Societies: ESC / ERS
DOI: 10.1093/eurheartj/ehac237
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What's New in 2022

Key updates from the 2015 ESC/ERS Guidelines:

Hemodynamic Definitions

PH is defined by right heart catheterization showing mean pulmonary artery pressure (mPAP) >20 mmHg at rest. Further classification depends on pulmonary artery wedge pressure (PAWP) and pulmonary vascular resistance (PVR).

Key Hemodynamic Parameters

Parameter Normal Abnormal
mPAP <20 mmHg >20 mmHg
PAWP ≤15 mmHg >15 mmHg
PVR <2 WU ≥2 WU
Cardiac Output (CO) 4-8 L/min <4 L/min

Pre-capillary vs. Post-capillary PH

PH Classification (Five Groups)

Group 1: Pulmonary Arterial Hypertension (PAH)

Group 2: PH Associated with Left Heart Disease

Group 3: PH Associated with Lung Diseases/Hypoxia

Group 4: Chronic Thromboembolic PH (CTEPH)

Group 5: PH with Unclear/Multifactorial Mechanisms

Diagnostic Approach

Three-Step Strategy

Step 1: Suspicion

Primary care evaluation: history, physical exam, ECG, BNP/NT-proBNP, O₂ saturation. Identify symptoms (dyspnea, fatigue, syncope) and risk factors.

Step 2: Detection

Echocardiography: TRV measurement, assess for PH signs. CPET, V/Q scan, CTPA, biomarkers as indicated.

Step 3: Confirmation

Right heart catheterization (RHC): Gold standard. Risk stratification at diagnosis using four-strata model.

Echocardiographic Probability of PH

Right Heart Catheterization & Hemodynamics

RHC Protocol

Measure RAP, PAP systolic/diastolic/mean, PAWP, CO. Calculate PVR and TPR. RHC comprises complete hemodynamic assessment following standardized protocols.

Vasoreactivity Testing

Identify acute responders (mPAP reduction ≥10 mmHg to ≤40 mmHg with unchanged/increased CO) who may benefit from high-dose CCB monotherapy.

  • Inhaled nitric oxide (iNO): Preferred (5-10 ppm, 5-10 min)
  • IV epoprostenol: Alternative (2-12 ng/kg/min)
  • Inhaled iloprost: Alternative option

Hemodynamic Measures

Risk Stratification

Use four-strata model (low/intermediate-low/intermediate-high/high) based on clinical, biochemical, imaging, and hemodynamic parameters.

Key Risk Variables

  • Clinical: WHO functional class, symptom progression, syncope
  • Exercise: 6MWD, CPET parameters (peak VO₂, VE/VCO₂ slope)
  • Biomarkers: BNP/NT-proBNP levels
  • Imaging: RA area, TAPSE, RV FAC, RVEF, SVI (echocardiography/cMRI)
  • Hemodynamics: RAP, mPAP, PAWP, CI, SVI

Categories: Low-risk <5% annual mortality; intermediate-low 5-20%; intermediate-high 20-40%; high-risk >40%.

PAH-Specific Therapies

Calcium Channel Blockers (CCBs)

Class I in acute responders. Nifedipine 120-240 mg/day, diltiazem 360+ mg/day, amlodipine 5-20 mg/day.

Endothelin Receptor Antagonists (ERAs)

Phosphodiesterase-5 Inhibitors (PDE5is)

Soluble Guanylate Cyclase (sGC) Stimulator

Prostacyclin Analogues

Initial Combination Therapy (High-Risk PAH)

Low/intermediate-risk: ERA + PDE5i (Class I)
High-risk: ERA + PDE5i + IV prostacyclin analogue (Class IIa)
Ambrisentan + tadalafil combination recommended (Class I)

Sequential Escalation (Follow-up)

Add macitentan to PDE5i/sGC (Class IIa)
Add selexipag to ERA/PDE5i (Class IIa)
Add IV/SC treprostinil or oral iloprost (Class IIa)

Group 2: PH Associated with Left Heart Disease

PH from elevated pulmonary venous pressure (HFrEF, HFpEF, valvular disease). Optimize underlying cardiac disease management (ACEi/ARB, beta-blockers, diuretics).

Don't:

  • Routinely use PAH-specific drugs without strong indication
  • Apply PAH algorithms to isolated post-capillary PH without addressing underlying cause

Group 3: PH Associated with Lung Disease/Hypoxia

COPD (~20% have severe PH), ILD, hypoxia-related (high altitude, sleep apnea). Optimize lung disease treatment; long-term O₂ if PaO₂ <8 kPa or SaO₂ <90%.

Management Approach

Treat underlying lung disease: bronchodilators, corticosteroids
Long-term oxygen therapy for hypoxemia
Screen for sleep apnea and treat if present
PAH-specific drugs: limited evidence; specialist consultation

Chronic Thromboembolic PH (CTEPH) – Group 4

Suspected in post-PE patients with persistent dyspnea. Confirm with V/Q imaging (mismatched defects) and RHC.

Diagnostic Algorithm

Treatment Options

Do:

  • Refer all suspected CTEPH to expert centres for operability assessment
  • Continue lifelong anticoagulation
  • Consider riociguat for inoperable CTEPH or persistent post-PEA PH

Special Populations

Pregnancy & Childbearing Potential

Pregnancy is contraindicated in PAH due to high maternal mortality risk. Counsel women; use reliable contraception; provide psychological support.

Congenital Heart Disease (CHD)-Associated PAH

PAH developing in ASD, VSD, PDA, complex CHD. Early surgical correction improves outcomes. Treatment similar to idiopathic PAH but individualized.

Portopulmonary Hypertension

PAH in advanced liver disease/portal hypertension. TIPS or transplantation may be considered; PAH-specific drugs explored cautiously.

CTD-Associated PAH

Common in SSc; often diagnosed late. Screen annually. Similar treatment to idiopathic PAH.

Pediatric PAH

Risk stratification and treatment algorithms adapted for age. Weight-based dosing essential.

Key Do's & Don'ts

Do:

  • Refer suspected PH to specialist centres early
  • Perform RHC in all suspected PAH for hemodynamic confirmation
  • Risk-stratify at diagnosis and reassess every 3-6 months
  • Screen for PAH in CTD, CHD, HPAH family history, portal HTN
  • Counsel women about contraception and pregnancy risks
  • Optimize general measures: exercise, oxygen, psychosocial support
  • Vaccinate against influenza and pneumococcal disease
  • Refer for lung transplantation if meeting advanced criteria

Don't:

  • Delay referral to PH centres in suspected PH
  • Initiate PAH drugs without RHC confirmation
  • Use PAH-specific drugs routinely in Group 2 or 3 without specialist input
  • Skip vasoreactivity testing in responder candidates
  • Recommend pregnancy in women with PAH
  • Overlook screening for secondary causes
  • Use ACEi/ARB/SGLT2i/beta-blockers in PAH without careful indication
  • Forget routine clinical, biomarker, and hemodynamic monitoring

Clinical Calculators & Tools

Adult GFR

Estimate glomerular filtration rate for renal function

Creatinine Clearance

Cockroft-Gault renal clearance estimation

Wells PE Score

PE probability assessment

Revised Geneva Score

PE probability using clinical data

PESI Score

PE risk stratification and mortality

MAGGIC HF Score

Heart failure mortality prognostication

H₂FPEF Score

HFpEF diagnostic algorithm

EuroSCORE II

Cardiac surgery operative risk

CHA₂DS₂-VASc

Atrial fibrillation stroke risk

Recommendation Classes & Evidence Levels

Class of Recommendation

Class Definition Wording
Class I Beneficial, useful, effective Is recommended / is indicated
Class IIa Weight of evidence favors usefulness Should be considered
Class IIb Less well established May be considered
Class III Not beneficial or harmful Is not recommended

Level of Evidence

Level Definition
Level A Multiple RCTs or meta-analyses
Level B Single RCT or large non-randomized studies
Level C Expert consensus / observational studies