Pulmonary Hypertension

Question 1

What is pulmonary hypertension defined using?

Answer 1

Mean pulmonary arterial pressure >20mmHg

Question 2

What are the causes of pulmonary hypertension? (5 groups)

Answer 2

1. Idiopathic pulmonary hypertension and connective tissue disorders e.g. SLE
2. Left heart failure (e.g. MI or systemic htn)
3. Chronic lung disease (e.g. COPD or pulmonary fibrosis)
4. Pulmonary vascular disease (e.g. PE)
5. Miscellaneous (e.g. sarcoidosis, glycogen storage disease + haematological disorders)

Question 3

What are the signs and symptoms of pulmonary hypertension? (6)

Answer 3

Sob

• Syncope (loss of consciousness)
• Tachycardia
• Raised jugular venous pressure (JVP)
• Hepatomegaly
• Peripheral oedema

Question 4

What ecg changes are seen in pulmonary hypertension? (4)

Answer 4

• P pulmonale (peaked P waves)
• Right ventricular hypertrophy (tall R waves in V1 and V2 and deep S waves in V5 and V6)
• Right axis deviation
• Right bundle branch block

Question 5

What cxr changes are seen in pulmonary hypertension? (2)

Answer 5

○ Dilated pulmonary arteries
○ Right ventricular hypertrophy

Question 6

Aside from ecg and cxr what other investigations can be done in pulmonary hypertension? What do they show? (2)

Answer 6

§ Raised NT‑proBNP blood test result indicates right ventricular failure
§ Echocardiogram can be used to estimate the pulmonary artery pressure

Question 7

What is the prognosis of idiopathic pulmonary hypertension?

Answer 7

2-3 years

Question 8

What medical managements can be used in pulmonary hypertension? (4)

Answer 8

• Calcium channel blockers
• Intravenous prostaglandins (e.g., epoprostenol)
• Endothelin receptor antagonists (e.g., macitentan)
• Phosphodiesterase-5 inhibitors (e.g., sildenafil)

Question 9

How is secondary pulmonary hypertension managed?

Answer 9

Treating underlying causes e.g. PE, COPD, sle