Pulmonary Hypertension Flashcards
What is 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.
How many causes are there?
5
Group 1 cause
Primary pulmonary hypertension or connective tissue disease such as systemic lupus erythematous (SLE)
Group 2 cause
Left heart failure usually due to myocardial infarction or systemic hypertension
Group 3 cause
Chronic lung disease such as COPD
Group 4 casue
ulmonary vascular disease such as pulmonary embolism
Group 5 cause
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.
Investigations needed
ECG
CXR
Bloods
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
P pulmonale
CXR changes
Dilated pulmonary arteries
Right ventricular hypertrophy
Blood test results
A raised NT-proBNP blood test result indicates right ventricular failure
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.
How can primary pulmonary hypertension be treated?
Treat underlying condition
Reduction of pulmonary vascular resistance: Long term oxygen therapy, Nifedipine, Sildenafil, Prostacycline analogues, Bosentan (endothelin receptor antagonist)
Manage heart failure
Heart-lung transplant for selected cases
IV prostanoids (e.g. epoprostenol) Endothelin receptor antagonists (e.g. macitentan) Phosphodiesterase-5 inhibitors (e.g. sildenafil)
How can secondary pulmonary hypertension?
Secondary pulmonary hypertension is managed by treating the underlying cause such as pulmonary embolism or SLE.
