Pulmonary Hypertension

Question 1

Causes of pulmonary hypertension

Answer 1

• Split into 5 groups:
  
  • 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 2

Signs and symptoms of pulmonary HTN

Answer 2

• SOB
• Syncope
• Tachycardia
• Raised JVP
• Hepatomegaly
• Peripheral oedema

Question 3

Investigation of pulmonary HTN

Answer 3

• ECG changes
  
  • RCH (seen as larger R waves on right sided chest leads [V1-3] and S aves on left sided chest leads [V4-6]
• CXR
  
  • Dilater pulmonary arteries
  • RVH
• Raised NT-proBNF indicates right ventricular failure
• ECHO to estimate pulmonary pressure

Question 4

Management of pulmonary HTN

Answer 4

• Prognosis poor (30-40% survival at 5 years from diagnosis)
• Primary pulmnary HTN
  
  • IV prostanoids
  • Endothelin receptor antagonists
  • Phosphodiesterase-5 inhibitors (i.e. sildenafil)
• Secondary pulmonary HTN
  
  • Treat underlying cause
• Supportive treatment for complications like respiratory failure, arrhythmias and HF