Pulmonary Hypertension

Question 1

Define Pulmonary Hypertension

Answer 1

Consistently increased pulmonary arterial pressure (>20mmHg) in resting conditions

Question 2

Aetiology of pulmonary hypertension

Answer 2

Primary: Idiopathic
Secondary:
- Left heart disease e.g. mitral valve disease, LV failure, LA myxoma/thrombosis
- Chronic lung disease e.g. COPD
- Recurrent pulmonary emboli
- Increased pulmonary blood flow e.g. ASD, VSD, patent ductus arteriosus
- Connective tissue disease e.g. SLE , systemic sclerosis
- Drugs e.g. amiodarone

Question 3

What are the WHO groups of pulmonary hypertension

Answer 3

1. Narrowing of blood vessels → Right heart hypertrophy → Right Heart Demand > Supply → Right Heart Failure
2. Left heart failure →Back pressure through pulmonary system → Pulmonary oedema + Pleural effusion
3. Hypoxia → Hypoxic pulmonary vasoconstriction (stop blood flow to damaged lung with little O2) → Pulmonary HTN
4. Blood vessel blocked/narrowed by blood clot → Narrowed vessel → Pulmonary HTN

Question 4

Risk factors for pulmonary hypertension

Answer 4

Obesity
Family history
Cardiopulmonary pathology
Liver disease
HIV
Recurrent PE
Cocaine
High altitude

Question 5

Symptoms of pulmonary hypertension

Answer 5

SOB on exertion
Chest pain
Syncope
Fatigue
Symptoms of underlying cause e.g. chronic cough in COPD

Question 6

Signs of pulmonary hypertension

Answer 6

Raised JVP (prominent a-wave)
Left parasternal heave (left ventricular hypertrophy)
Auscultation:
- Loud pulmonary component of S2
- S3 or S4
- Pulmonary regurgitation → Graham-Steel murmur (early diastolic)
- Tricuspid regurgitation → Large cv-wave + pansystolic murmur
Severe → Right heart failure
Signs of underlying condition

Question 7

Investigations for pulmonary hypertension

Answer 7

ECG:
- RVH: Right axis deviation, prominent R wave (V1), T inversion V1-V2
- RA enlargement: peaked P wave lead II (P-pulmonale)
- COPD: limb leads have low voltage (R <5mm)

CXR:
- Cardiomegaly (RV enlargement, RA dilation)
- Prominent main pulmonary arteries
- Signs of the cause (COPD, calcified metal valves)
Echo: ?RVH, dilatation
CT chest: ?pulmonary arteries
Lung function testing, V/Q
Cardiac catheterisation: severity

Question 8

Management for pulmonary hypertension

Answer 8

Medical: - Treat secondary cause
For primary pulmonary HTN, consider:
- Anticoagulant
- CCB
- Prostacycline analogue
- Endothelial receptor antagonist (blocks vasoconstriction)
- Phosphodiesterase inhibitor (promote SM relaxation → pulmonary BP)

Surgical: Heart / Lung transplant

Question 9

Complications of pulmonary hypertension

Answer 9

Right heart failure (cor pulmonale)
Arrhythmias (AF, VT, VF)
Sudden death