Pulmonary hypertension

Question 1

What is pulmonary hypertension?

Answer 1

Increased resistance and pressure of blood in the pulmonary arteries.

Question 2

1) What is the group 1 cause of pulmonary hypertension?
2) What is the group 2 cause of pulmonary hypertension?
3) What is the group 3 cause of pulmonary hypertension?
4) What is the group 4 cause of pulmonary hypertension?
5) What is the group 5 cause of pulmonary hypertension?

Answer 2

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

Question 3

1) What is the main presenting symptom?
2) Name another sign or symptom

Answer 3

1) Shortness of breath
2) Syncope, tachycardia, raised JVP, hepatomegaly, peripheral oedema

Question 4

Name a potential ECG change that might be seen due to the pressure on the right side of the heart

Answer 4

• Right ventricular hypertrophy seen as larger R waves on the right sided chest leads (V1-3) and S waves on the left sided chest leads (V4-6)
• Right axis deviation
• Right bundle branch block

Question 5

Name another investigation that may be done

Answer 5

• Chest x-ray
• Echocardiogram

Question 6

A raised what in blood tests indicates right sided heart failure?

Answer 6

NT-proBNP

Question 7

Name a medication that can be used to treat primary pulmonary hypertension

Answer 7

• IV prostanoids (e.g. epoprostenol)
• Endothelin receptor antagonists (e.g. macitentan)
• Phosphodiesterase-5 inhibitors (e.g. sildenafil)

Question 8

Name another feature of the management of pulmonary hypertension

Answer 8

• Management of underlying cause
• Supportive treatment of any complications