Pulmonary Hypertension

Question 1

What is pulmonary hypertension?

Answer 1

increased resistance and pressure of blood in the pulmonary arteries.

Question 2

what does increased pressure and resistance in the pulmonary arteries lead to?

Answer 2

strain on the right side of the heart trying to pump blood through the lungs

also causes a back pressure of blood into the systemic venous system

Question 3

What are the 5 groups which can cause pulmonary hypertension?

Answer 3

Group 1 – Primary pulmonary hypertension or connective tissue disease such as systemic lupus erythematous (SLE)
Group 2 – Left heart failure usually due to myocardial infarction or systemic hypertension
Group 3 – Chronic lung disease such as COPD
Group 4 – Pulmonary vascular disease such as pulmonary embolism
Group 5 – Miscellaneous causes such as sarcoidosis, glycogen storage disease and haematological disorders

Question 4

What is the key presentation of pulmonary htn?

What are the other symptoms?

Answer 4

Shortness of breath

others

• syncope
• tachycardia
• raised JVP
• hepatomegaly
• peripheral oedema

Question 5

What investigations would you do?

Answer 5

1. ECG
2. CXR
3. NT-proBNP - indicates right heart failure
4. ECHO - to estimate pulmonary artery pressure

Question 6

What are ECG findings of pulmonary hypertension?

Answer 6

Right heart strain

1. Right ventricular hypertrophy (larger R waves on right sided chest lead - V1-V3 and larger S waves on left sided leads V4-V6)
2. Right Bundle Branch Block
3. Right axis deviation

Question 7

What do you expect to see on the chest x-ray in pulmonary hypertension?

Answer 7

1. dilated pulmonary arteries

2. right ventricular hypertrophy

Question 8

What is the prognosis of pulmonary hypertension?

Answer 8

poor. 30-40% survival rate at 5 years, improves to 60-70% where specific treatment possible

Question 9

What is the management of pulmonary hypertension?

Answer 9

primary pulmonary hypertension

1. IV prostanoids (prostoglands - expand blood vessels)
2. Endothelin receptor antagonists
3. Phosphodiesterase-5-inhibitors (sildenafil)

Secondary pulmonary hypertension is managed by treating the underlying cause such as pulmonary embolism or SLE.

Supportive treatment for complications such as respiratory failure, arrhythmias and heart failure.