Pulmonary Hypertension Flashcards
What is pulmonary hypertension?
An increase in mean pulmonary arterial pressure* (normally around 15mmHg) which is associated with a number of conditions.
*resistance and pressure of blood in the pulmonary arteries
What does an increase in pressure and resistance cause?
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.
What are the causes of pulmonary hypertension?
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
Signs and symptoms
Shortness of breath is the main presenting symptom.
Other signs and symptoms are:
Syncope
Tachycardia
Raised JVP
Hepatomegaly
Peripheral oedema.
Gold standard investigation and result
Right heart catheterisation (gold standard) shows mean pulmonary artery pressure >25mmHg
ECG changes
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
X-ray changes
Dilated pulmonary arteries
Right ventricular hypertrophy
Prognosis
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 treatment
IV prostanoids (e.g. epoprostenol)
Endothelin receptor antagonists (e.g. macitentan)
Phosphodiesterase-5 inhibitors (e.g. sildenafil)
Secondary pulmonary hypertension treatment
Secondary pulmonary hypertension is managed by treating the underlying cause such as pulmonary embolism or SLE.
