Pulmonary Hypertension

Pulmonary hypertension is increased resistance and pressure of blood in the pulmonary arteries. Increasing the pressure and resistance in the pulmonary arteries causes strain on the right side of the heart trying to pump blood through the lungs. This also causes a back pressure of blood into the systemic venous system.


The causes of pulmonary hypertension can split into 5 groups:

  • Group 1Primary 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


Signs and Symptoms

Shortness of breath is the main presenting symptom.

Other signs and symptoms are:

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



ECG Changes

The right sided heart strain causes ECG changes such as:

  • Right ventricular 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)
  • Right axis deviation
  • Right bundle branch block


Chest Xray Changes

  • Dilated pulmonary arteries
  • Right ventricular hypertrophy


Other investigations

  • A raised NT-proBNP blood test result indicates right ventricular failure
  • Echo can be used to estimate pulmonary artery pressure



The prognosis is quite poor with a 30-40% 5-year survival from diagnosis. This can increase to 60-70% where specific treatment is possible.

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 pulmonary hypertension is managed by treating the underlying cause such as pulmonary embolism or SLE.

Supportive treatment for complications such as respiratory failure, arrhythmias and heart failure.


Last updated March 2019
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