Pulmonary Hypertension

Question 1

What is pulmonary hypertension?

Answer 1

Increased resistance and pressure of blood in the pulmonary arteries

Question 2

What is there an increase of with pulmonary hypertension?

Answer 2

There is an increase in mean pulmonary arterial pressure (normally around 15mmHg)

Question 3

What can an increase in pulmonary arterial pressure result in?

Answer 3

Strain on the right side of the heart

Question 4

How does increased pulmonary arterial pressure result in right heart strain?

Answer 4

Heart trying to pump blood through the lungs

Question 5

What effect can increased pulmonary arterial pressure have on the venous system?

Answer 5

Can cause a back pressure of blood into the systemic venous system

Question 6

What is the main presenting symptom of pulmonary hypertension?

Answer 6

Shortness of breath

Question 7

What are other possible presenting symptoms of pulmonary hypertension?

Answer 7

Syncope
Tachycardia
Raised JVP
Peripheral oedema

Question 8

What investigations would you carry out in someone with increased pulmonary arterial pressure?

Answer 8

ECG (MAIN)
Echocardiogram
Chest x-ray
Right heart catheterisation

Question 9

What ECG changes would you see in someone presenting with pulmonary hypertension?

Answer 9

Right ventricular hypertrophy
Right axis deviation
Right bundle branch block

Question 10

What changes might you see on a chest x-ray of someone with pulmonary hypertension?

Answer 10

Dilated pulmonary arteries
Right ventricular hypertrophy

Question 11

What would right heart catheterisation show in someone with pulmonary hypertension?

Answer 11

Mean pulmonary artery pressure >25mmHg

Question 12

What can the causes of pulmonary hypertension be divided into?

Answer 12

5 groups

Question 13

What is group 1 pulmonary hypertension?

Answer 13

Primary pulmonary hypertension or
Connective tissue disease- SLE

Question 14

What is group 2 pulmonary hypertension?

Answer 14

Left heart failure usually due to myocardial infarction or systemic hypertension

Question 15

What is group 3 pulmonary hypertension?

Answer 15

Chronic lung disease- COPD

Question 16

What is group 4 pulmonary hypertension?

Answer 16

Pulmonary vascular disease- pulmonary embolism

Question 17

What is group 5 pulmonary hypertension?

Answer 17

Miscellaneous causes such as sarcoidosis, glycogen storage disease and haematological disorders

Question 18

How can primary pulmonary hypertension be treated?

Answer 18

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

Question 19

How can secondary pulmonary hypertension be managed?

Answer 19

Treat the underlying cause such as pulmonary embolism or SLE.

Question 20

How can pulmonary vascular resistance be reduced?

Answer 20

Long term oxygen therapy, Nifedipine, Sildenafil, Prostacycline analogues, Bosentan (endothelin receptor antagonist)