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.
Interactive Decision Tree
Algorithm Steps
- ▶Start
Suspected Pulmonary Hypertension
Symptoms: Dyspnea, fatigue, syncope, RV failure signs
- ●Action
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
- ◆Decision
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
- ●Action
Low Probability
Consider alternative diagnoses
- PH unlikely if no other signs
- Investigate other causes of symptoms
- Follow up if symptoms persist
- ●Action
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
- ◆Decision
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
- ◆Decision
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
- ●Action
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
- ●Action
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
- ●Action
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
- ◆Decision
Response at 3-6 Months?
Reassess risk status
- Achieving low-risk status = goal
- If not at goal: Escalate therapy
- Add prostacyclin pathway agent
- ●Action
Escalate Therapy
Not at treatment goal
- Add prostacyclin analogue
- Selexipag (oral)
- Treprostinil (inhaled, SC, IV)
- Epoprostenol (IV)
- Consider transplant referral
- ✓Outcome
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
- ●Action
Group 2: Left Heart Disease
Treat underlying cardiac condition
- Optimize HF therapy
- PAH-specific drugs NOT recommended
- Diuretics for congestion
- Consider MitraClip, LVAD, transplant
- ●Action
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
Global: ESC/ERS guidelines are reference standard for PH management
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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.
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