Pulmonary Hypotension

Question 1

What is pulmonary hypertension?

Answer 1

Increased resistance and pressure of blood in the pulmonary arteries

Causes strain on the right side of the heart and back pressure into systemic venous system

Question 2

Causes of pulmonary hypertension (5)

Answer 2

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 3

Signs and symptoms

Answer 3

SOB

Syncope
Tachycardia
Raised JVP
Hepatomegaly
Peripheral oedema

Question 4

Investigations for pulmonary hypertension

Answer 4

ECG
CXR
NT-proBNP (RVF)
Echo (estimate pulmonary artery pressure)

Question 5

ECG findings in pulmonary hypertension

Answer 5

Right ventricular hypertrophy - larger R waves v1-3, S waves V4-V6

Right axis deviation

RBBB

Question 6

CXR findings in pulmonary hypertension

Answer 6

Dilated pulmonary arteries

Right ventricular hypertrophy

Question 7

Prognosis of pulmonary hypertension

Answer 7

30-40% 5-year survival from diagnosis

Can increase to 60-70% where specific treatment is possible

Question 8

Management of pulmonary hypertension

Answer 8

Primary pulmonary hypertension can be treated with:

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

Secondary - Treat underlying cause