Pulmonary hypertension Flashcards
What is PH?
Pulmonary hypertension is increased resistance and pressure of blood in the pulmonary arteries.
What are the causes?
The causes of pulmonary hypertension can 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
What are the signs and symptoms?
Syncope
Tachycardia
Raised JVP
Hepatomegaly
Peripheral oedema.
What are the investigations?
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
What is the management?
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.
