What is PH?
Pulmonary Hypertension (PH) is an all-encompassing term used to describe an increased blood pressure in the lungs. This term spans across a number of conditions that are grouped into 5 key categories:
- Group 1: Pulmonary Arterial Hypertension (PAH)
- Group 2: PH due to left heart disease
- Group 3: PH due to lung disease (e.g. COPD, Pulmonary Fibrosis)
- Group 4: CTEPH
- Group 5: Pulmonary Hypertension due to other causes
The symptoms for each of these types are similar, and include breathlessness, fatigue, dizziness, chest pain, and coughing up blood.
What is PAH?
Pulmonary Arterial Hypertension (PAH) is one type of PH caused by the narrowing or blockage of the blood vessels (arteries) that carry blood from the heart to the lungs.
PAH can occur on its own with no known cause (idiopathic), but it can also be genetic or associated with another condition such as connective tissues diseases, congenital heart disease, blood clots, or several other rare causes. Investigating the underlying cause of your PAH will help your treating doctor determine the most suitable treatment options available.
How is PAH diagnosed?
Pulmonary Arterial Hypertension has many common symptoms associated with other heart and lung diseases. Therefore, a few different tests may be performed to accurately diagnose PAH, including:
- Lung function tests
- Chest x-ray
- Echocardiogram (ultrasound of the heart)
- This test looks at the pressures of the heart and how the heart is pumping or functioning.
- Right heart catheterisation
- This test involves threading a wire through either the groin, wrist, or neck which directly measures pressure in the heart and lungs.
- This usually confirms a PAH diagnosis and can also indicate why the PAH has occurred
What are the treatment options for PAH?
Although there is no current cure for PAH, studies have shown that early treatment is important to help improve how you feel and to keep your PAH well managed for many years.
Usually, you will start with a tablet medication, of which there are three classes. In most patients, these tablets will lower the pressure of the heart, reduce your breathlessness and improve your wellbeing.
In some cases, people living with PAH may be recommended other treatment options such as intravenous therapy, oxygen therapy, or in rare cases a lung transplant.
What is CTEPH?
CTEPH, or chronic thromboembolic disease, is another type of PH caused by long term blood clots in the lungs.
When most people get blood clots in the lungs, their body dissolves the blood clots and blood thinners stop any new clots forming. In CTEPH however, the body doesn’t dissolve these blood clots, and after some time this can block the vessels in the lungs and cause a build-up of pressure, which causes PH.
How is CTEPH Diagnosed?
In order to accurately diagnose CTEPH, the presence of PH first needs to be confirmed, as well as the presence of blood clots. Therefore, once your treating doctor has administered the diagnostic measures for PH, they would order additional tests to investigate for CTEPH.
One test is a ventilation-perfusion (VQ) scan, which helps your specialist identify if you have blood clots in your lungs.
If blood clots are suspected, the next test may be a CT pulmonary angiogram, where dye contrast is put through the vessels to see whether they are narrowed due to blood clots, or whether the blood is flowing through.
The other test is a formal pulmonary angiogram, which can provide more accurate details as to where the clots are, and in turn help your treating specialist identify suitable treatment options.
What are the treatment options for CTEPH?
There are 3 main treatment options for CTEPH, which is dependent on the location of the clot, other underlying medical conditions, and the patients’ preferences.
The first option is surgery, which involves opening the chest and removing all blood clots. It is a major surgery that works well when the clot is close to the entrance and easily accessed, but it isn’t as effective when the clot is deep in the lungs.
Another option is a Balloon Pulmonary Angioplasty (BPA), which opens the vessels, reduces the resistance and allows the blood to flow through.
If surgery or a BPA is not appropriate, medication can also be an option. While it won’t get rid of the blood clot, it will reduce the pressure in the lungs and help improve both quality of life and life expectancy.
However, all people living with CTEPH should be put on blood thinners to prevent new blood clots from forming in the future, even after undergoing surgery or BPA.
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