It can be Primary pulmonary hypertension in which no cause is found or it can be secondary to heart/lung/blood etc issues.
Cardiac diseases can be in the form of holes in the heart which can be associated with or without cyanosis. Acyanotic and cyanotic congenital heart diseases with large shunt lesions if not treated early, can lead to increase inpulmonary pressures (Pulmonary Hypertension/Eisenmenger Syndrome).
These heart diseases commonly are large VSD, PDA, AP window or can be cyanotic like TGA, TAPVC etc.
Diagnosis is done by clinical history and physical examination as well as by doing some tests. Test may include:
- Chest X-ray
- 6 minute walk test
- Right heart catheterization: This is an angiographic test that we do in cath lab. By this method we can measure pressure in the main pulmonary arteries and right ventricle accurately.
- Computerized tomography (CT) scan
- Magnetic resonance imaging (MRI)
- Blood tests
- Pulmonary function test
- Polysomnogram: help in diagnosing a sleep disorder such as obstructive sleep apnea.
- Ventilation/perfusion (V/Q) scan
- Open-lung biopsy. Rarely done
If pulmonary hypertension in early stages then we should correct the heart diseases as early as possible.
If pulmonary hypertension is in early to late stages then we should evaluate whether we can correct the heart disease or not.
Pulmonary hypertension if irreversible then cannot be cured.Only symptomatic relief can be provided by medications for maintaining good quality of life.
- Pulmonary vasodilators: These medicines causes increase in size of pulmonary vessels so decrease in lung pressures.
- Commonly used drugs are: Sildenafil, bosentan
- Others are:Epoprost, iloprostetc
- High-dose calcium channel blockers.
- Soluble guanylatecyclase (SGC) stimulator.
- Anticoagulants: Help in prevention of formation of blood clots within the small pulmonary arteries.
- Surgeries: Atrial septostomy, Transplantation.