Pulmonary Hypertension

Question 1

What is the difference between pulmonary and secondary hypertension?

Answer 1

Pulmonary hypertension is classified as idiopathic or primary when the cause is unknown. When pulmonary hypertension results from known risk factors or underlying diseases, it is classified as secondary pulmonary hypertension.

Question 2

What is pulmonary hypertension?

Answer 2

An increase in mean pulmonary arterial pressure >20mmHg under resting conditions.

Question 3

What are the causes/ risk factors of pulmonary hypertension?

Answer 3

• Idiopathic 
• CTD (connective tissue disease)
• Drugs (amiodarone) 
• Parenchymal lung disease: COPD, Chronic Asthma, Interstitial lung disease, bronchiectasis, cystic fibrosis
• Pulmonary vascular disease: Idiopathic pulmonary hypertension, pulmonary vasculitis, pulmonary embolism, portal hypertension
• Hypoventilation: Sleep apnoea, kyphosis/scoliosis, neuromuscular conditions (e.g. myasthenia gravis)
• Left Heart Disease: Mitral stenosis, mitral regurgitation, left ventricular failure

Question 4

Summarise the epidemiology of pulmonary hypertension

Answer 4

Idiopathic pulmonary hypertension is RARE
More common in severe respiratory and cardiac disease

Question 5

What presenting symptoms of pulmonary hypertension can be found in the history?

Answer 5

• Progressive breathlessness
• Weakness/tiredness
• Exertional dizziness and syncope
• LATE STAGE - oedema and ascites
• Angina and tachyarrhythmia

Question 6

What signs of pulmonary hypertension can be found on physical examination?

Answer 6

• Right ventricular heave 
• Loud pulmonary second heart sound 
• Murmur - pulmonary regurgitation 
• Tricuspid regurgitation 
• Raised JVP 
• Peripheral oedema 
• Ascites 
• Palpation: Left parasternal heave (right ventricular hypertrophy).  

Question 7

What investigations are used to diagnose/ monitor pulmonary hypertension?

Answer 7

1. Transthoracic Echocardiogram → first-line in all patients with suspected PH
2. Right Heart Catheterisation → confirmatory test. Will show mPAP >20mmHg if PH, shows extent of disease
3. CXR - exclude other lung diseases/ right heart hypertrophy
4. ECG - right ventricular hypertrophy and strain
   - P pulmonale is a right atrial abnormality that is seen on ECG as tall, peaked P waves. It is a feature that can be seen on ECG in patients with pulmonary hypertension, as it is a cause of right atrial enlargement.
5. Pulmonary function tests
6. LFTs - liver damage –> portal hypertension
7. Lung biopsy - interstitial lung disease
8. Echocardiography - assess right ventricular function

Question 8

How is pulmonary hypertension managed?

Answer 8

1. Calcium Channel Blockers → first-line pulmonary vasodilator therapy for patients with idiopathic pulmonary hypertension and no signs of RHF
2. Lifestyle Changes → exercise, restrict sodium intake, avoid pregnancy
3. Anticoagulation → warfarin, adjust dose according to INR