Suspected Pulmonary Hypertension
Symptoms: Dyspnea, fatigue, syncope, RV failure signs
All Pathways
Pulmonary MedicineManagement
Pulmonary Hypertension Diagnosis and Management (ESC/ERS 2022)
Pulmonary Hypertension Diagnosis and Management (ESC/ERS 2022): Suspected Pulmonary Hypertension → Initial Workup → Echo PH Probability? → Low Probability.
Pathway Overview
15 steps
Algorithm Steps
15 total
01Start 02Action Initial Workup
Non-invasive assessment
- ECG: RVH, RAD, RBBB
- CXR: Enlarged PA, pruning of vessels
- TTE: Estimate PASP, RV function
- BNP/NT-proBNP: Elevated in RV dysfunction
- PFTs + DLCO: Identify lung disease
03Decision Echo PH Probability?
Based on TR velocity and other signs
- Low: TRV ≤2.8 m/s, no other signs
- Intermediate: TRV ≤2.8 + other signs OR TRV 2.9-3.4
- High: TRV >3.4 m/s OR TRV 2.9-3.4 + other signs
04Action Low Probability
Consider alternative diagnoses
- PH unlikely if no other signs
- Investigate other causes of symptoms
- Follow up if symptoms persist
05Action Further Testing Required
For intermediate/high probability
- V/Q scan: Rule out CTEPH
- HRCT: Assess lung parenchyma
- Sleep study if OSA suspected
- HIV, ANA, LFTs
- 6-minute walk test
06Decision Right Heart Catheterization
Confirm diagnosis and hemodynamics
- Essential for diagnosis and classification
- PH: mPAP >20 mmHg at rest
- Pre-capillary: PAWP ≤15, PVR >2 WU
- Post-capillary: PAWP >15
- Combined: PAWP >15 + PVR >2 WU
07Decision Classify PH Group
WHO Classification
- Group 1: PAH (idiopathic, heritable, drug-induced, CTD)
- Group 2: Left heart disease
- Group 3: Lung disease/hypoxia
- Group 4: CTEPH
- Group 5: Unclear/multifactorial
08Action Group 1: PAH
PAH-specific therapy indicated
- Refer to PH specialist center
- Risk stratification (low/intermediate/high)
- General measures: Diuretics, O2, anticoagulation (selected)
- Avoid pregnancy
- Vaccinations
09Action Risk Stratification (PAH)
Determine treatment intensity
- LOW RISK: WHO FC I-II, 6MWD >440m, NT-proBNP <300
- INTERMEDIATE: WHO FC III, 6MWD 165-440m
- HIGH RISK: WHO FC IV, 6MWD <165m, RV failure
- Consider: RA pressure, CI, SvO2
10Action Initial Combination Therapy
Based on risk stratification
- LOW/INTERMEDIATE: Oral combination
- - ERA (ambrisentan, bosentan, macitentan)
- - PDE5i (sildenafil, tadalafil) or sGC stimulator (riociguat)
- HIGH RISK: Add IV/SC prostacyclin
- - Epoprostenol IV, Treprostinil SC/IV
11Decision Response at 3-6 Months?
Reassess risk status
- Achieving low-risk status = goal
- If not at goal: Escalate therapy
- Add prostacyclin pathway agent
12Action Escalate Therapy
Not at treatment goal
- Add prostacyclin analogue
- Selexipag (oral)
- Treprostinil (inhaled, SC, IV)
- Epoprostenol (IV)
- Consider transplant referral
- Path rejoins step 11Shared downstream outcome
13Outcome Maintain & Monitor
At treatment goal
- Regular follow-up at PH center
- 6MWT and NT-proBNP every 3-6 months
- Annual RHC consideration
- Maintain medications
- Watch for deterioration
14Action Group 2: Left Heart Disease
Treat underlying cardiac condition
- Optimize HF therapy
- PAH-specific drugs NOT recommended
- Diuretics for congestion
- Consider MitraClip, LVAD, transplant
15Action Group 3: Lung Disease
Treat underlying lung condition
- Optimize COPD/ILD treatment
- Long-term oxygen therapy
- Pulmonary rehabilitation
- Consider inhaled treprostinil (approved for ILD-PH)
Guideline Source
2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension
Clinical Safety Information
Clinical Decision Support — Not a Substitute for Clinical Judgment
Individual patient factors may require deviation from these recommendations.
Known Limitations
- PAH-specific therapies require specialist initiation
- Risk assessment tools require multiple parameters
- Combination therapy protocols complex
- Does not cover all PH groups in detail
Applicable Regions
EUUSGlobal
Global: ESC/ERS guidelines are reference standard for PH management
Version 1Next review: 2027-01-01
Next steps
Finish the workflow by opening the most relevant calculator, then convert the session into a live account when you are ready.
Calculator
Pulmonary Embolism Severity Index (PESI)
30-day mortality risk stratification for acute pulmonary embolism
Open page
Compare
AttendMe.ai vs DynaMed
See how this pathway workflow compares against DynaMed.
Open page
Commercial
Start free
Run the pathway in a live AttendMe account with citations and tracked usage.
Start free
Related Resources
Frequently Asked Questions
What is the Pulmonary Hypertension Diagnosis and Management (ESC/ERS 2022)?
The Pulmonary Hypertension Diagnosis and Management (ESC/ERS 2022) is a management clinical algorithm for Pulmonary Medicine. It provides a structured decision tree to guide clinical decision-making, based on 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension.
What guideline is the Pulmonary Hypertension Diagnosis and Management (ESC/ERS 2022) based on?
This algorithm is based on 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension (DOI: 10.1093/eurheartj/ehac237).
What are the limitations of the Pulmonary Hypertension Diagnosis and Management (ESC/ERS 2022)?
Known limitations include: PAH-specific therapies require specialist initiation; Risk assessment tools require multiple parameters; Combination therapy protocols complex; Does not cover all PH groups in detail. Individual patient factors may require deviation from these recommendations.
Get AI-Powered Analysis Alongside This Algorithm
In AttendMe.ai, the Pulmonary Hypertension Diagnosis and Management (ESC/ERS 2022) appears automatically when your clinical question matches — alongside evidence from 3M+ peer-reviewed articles.
Try AttendMe Free