Pulmonary Hypertension

Question 1

What is pulmonary hypertension?

Answer 1

Increased resistance and pressure of blood in the pulmonary arteries (>25mmHg), causing strain on the right side of the heart trying to pump blood through the lungs, which also results in back pressure of blood into the systemic venous system

Question 2

What is Cor Pulmonale?

Answer 2

Right ventricular enlargement secondary to pulmonary disease

Question 3

What are the causes of pulmonary hypertension?

Answer 3

Primary

Connective tissue disease

Left heart failure

Chronic lung disease, such as COPD

Pulmonary vascular disease, such as PE

Sarcoidosis

Question 4

What causes chronic thromboembolic pulmonary hypertension (CTEPH)?

Answer 4

Arises in patients with chronic pulmonary embolism

Question 5

Describe the presentation of pulmonary hypertension

Answer 5

Dyspnoea

Cough

Syncope

Right ventricular heave

>JVP

Periphral oedema

Hepatomegaly

Loud S2 sound

Fatigue

Angina, due to right ventricle ischaemia

Question 6

What investigations are used in pulmonary hypertension diagnosis?

Answer 6

ECG

• RV 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)

CXR

• Dilated pulmonary arteries
• Right ventricular hypertrophy

ECHO

• RV and RA enlargement

CT Pulmonary Angiogram

Question 7

What is the management of primary pulmonary hypertension?

Answer 7

IV prostanoids

• Epoprostenol

Endothelin Receptor Antagonist

• Macitentan

Phosphodiesterase-5 inhibitors

• Sildenafil

If secondary, treat the underlying cause