Pulmonary Hypertension (PH)
Article status: Temporary high-yield summary
- This article will be fully reviewed, expanded, and referenced in due course
- Current content focuses on core principles and exam-relevant concepts
Aetiology
Left-heart disease is the leading cause of PH, followed by chronic lung disease, particularly COPD.
There are 5 groups of causes:
| Group | PH type | Important / common causes |
|---|---|---|
| 1 – Pulmonary arterial hypertension | Pre-capillary | Caused by pulmonary vascular disease
|
| 2 – PH due to left-heart disease | Post-capillary | Most common cause of PH overall
|
| 3 – PH due to lung disease | Pre-capillary | 2nd most common group of PH
|
| 4 – Chronic thromboembolic PH (CTEPH) | Pre-capillary | Due to unresolved pulmonary emboli |
| 5 – Unclear / multifactorial | Mixed | Examples include:
|
Clinical Features
Key symptoms:
- Exertional dyspnoea (most common)
- Reduced exercise tolerance / fatigue
- Chest pain
- Syncope / pre-syncope (esp. on exertion)
Signs of right-sided heart failure:
- Raised JVP
- Parasternal heave (from RV hypertrophy)
- Auscultation
- Loud P2
- Tricuspid regurgitation murmur (pansystolic murmur on the lower parasternal edge – louder on inspiration)
- Clear chest sounds (unless there is underlying lung disease)
- Peripheral oedema
- Ascites / congestive hepatomegaly
Investigation and Diagnosis
Initial evaluation:
- ECG, chest X-ray
- Basic bloods (including BNP/NT-proBNP)
Investigations:
| Purpose | Test | Key suggestive finding |
|---|---|---|
| 1st line screening | Trans-thoracic echocardiography | Provides an indirect estimate of pulmonary artery pressure:
|
| Further investigations | Lung function test with DLCO – ALL patients |
|
Screening for CTEPH:
|
|
|
| Assess for parenchymal lung disease – CT chest |
|
|
| Gold standard (for diagnosis and classification) | Right heart catheterisation | Key diagnostic finding: mean pulmonary arterial pressure ≥20 mmHg
Pulmonary artery wedge pressure is used to differentiate between pre- and post-capillary PH
|
Disclaimer:
Detailed interpretation of specialised investigations (e.g. echocardiographic parameters and right heart catheterisation measurements) is typically specialist-level. Only key high-yield principles are included here.
Management
Management depends on the group of PH:
| PH group | Management |
|---|---|
| 1 – Pulmonary arterial hypertension | Management depends on vasoreactivity testing:
|
| 2 – PH due to left-heart disease | Treat the underlying heart failure and cause of left-heart disease |
| 3 – PH due to lung disease | Optimise treatment of underlying cause |
| 4 – Chronic thromboembolic PH (CTEPH) | 1st line: pulmonary endarterectomy
Consider lifelong anticoagulation |
| 5 – Unclear / multifactorial | Treat underlying cause of PH |
For exam purposes, CCBs and other pulmonary vasodilators are only used to treat group 1 PH (pulmonary arterial hypertension), but NOT group 2 and 3 PH.
Exceptions do exist, but are out-of-scope for non-specialist level.