This month is Pulmonary Hypertension Awareness Month when charities launch campaigns to raise awareness about this progressive lung disease which is thought to affect around 1% of the global population (increasing to 10% for those aged over 65). As symptoms are non-specific, the disease is often misdiagnosed, which is why increasing awareness is so critical. While in most cases, no cure is currently available, early diagnosis can massively improve a patient’s outcome.
In simple terms, PH is a chronic and progressive disease, which causes high blood pressure in the vessels that carry blood to your lungs – or the pulmonary arteries. This is caused by these arteries becoming thick and stiff meaning they can’t expand as well to allow blood to pass through. This makes it much harder for the blood to flow to the lungs causing the pressure in the arteries to rise. As the pressure builds, the heart has to work harder to pump the blood to the lungs and over time starts to struggle with the extra workload, leading to the symptoms mentioned below and ultimately, if left untreated, to heart failure.
For the purposes of treatment, there are 5 different types of PH, each with its own range of causes. These causes can range from drugs or toxins to HIV infection, liver disease, or existing heart and lung disease. PH can also be brought on by blood, metabolic, or systemic, disorders. PH can in theory affect people of all ages, races and ethnic backgrounds though there are certain risk factors:
The first symptom most sufferers will notice is breathlessness during routine activities. You may also notice any of the following :
Unfortunately diagnosing high blood pressure in the pulmonary arteries isn’t as simple as a normal blood pressure test. PH can develop very slowly over time which is why it often gets misdiagnosed as asthma or other heart or lung conditions. If you’re suffering from any of the above symptoms, try entering them into the Isabel Symptom Checker before discussing them with your doctor. Or if you’re not sure where to seek care, try using Isabel’s Virtual Triage within the symptom checker tool, which combines the results you get from the symptom checker with your answers to 7 quick questions to help you decide where to seek care next. It’s important, however, to discuss these sort of symptoms with a doctor. If they suspect PH, they may refer you for an echocardiogram, which can measure the pressure in your pulmonary arteries. They may also suggest a Right Heart Catheterisation, which tends to offer the ultimate confirmation of a PH diagnosis. Other tests you might have are an ECG (electrocardiogram), lung function and breathing tests, or a range of scans.
Of the 5 different groups of PH, only one, group 4, can potentially be cured. Group 4 PH is caused by blood clots in the pulmonary arteries which, when removed by surgery, cause the patient to experience significantly fewer, or in some cases, no further symptoms. But for the other groups there is sadly no cure. However, there are treatments which can help reduce your symptoms. These depend on the original cause of the disease. For example your PH might be caused by low levels of oxygen in the blood, which in turn cause the pulmonary arteries to narrow. Oxygen treatment, which involves inhaling air containing a higher concentration of oxygen than normal, can be offered to help improve your symptoms. Some patients will need surgery to remove old blood clots or to carry out a procedure to help reduce blood pressure in the lung arteries. In severe cases, a lung transplant or heart-lung transplant might be recommended when medication is unable to help. In many cases, lifestyle changes will be advised to help improve symptoms. These can include:
Research for a cure is ongoing and with the continual improvements in diagnostic techniques and therapies, it is becoming easier to diagnose the disease much earlier. Not so long ago, survival of PH patients used to be as little as 3 to 5 years, and it could easily take 2 of those years just to reach a diagnosis. Nowadays, life expectancy is greatly improved with a recent study showing that over 62% of patients now survive 10 or more years after diagnosis. Early diagnosis is surely the key so if your symptoms are suspicious, don’t hesitate to discuss them with your doctor.