Pulmonary Arterial Hypertension: Not The High Blood Pressure You Know

By Dr Ruan Wen, Senior Consultant, Cardiology

If someone feels more tired than before, finds it harder to climb stairs, or sometimes experiences dizziness or chest tightness, these everyday symptoms should not be ignored. They could be early signs of a condition called pulmonary arterial hypertension (PAH), which is a type of pulmonary hypertension (PH). 

Understanding Pulmonary Hypertension and Its Types

Pulmonary hypertension is not a single disease. It is an umbrella term that includes several different types of conditions.

PAH is a unique type of PH (Group 1), and it is a disease of the arteries in the lungs. The cause may be unknown, inherited or linked to conditions like lupus. The most common form of PH is due to left heart disease (Group 2). This happens when heart failure or valve disease causes blood to back up into the lungs. Another type of PH is caused by lung disease (Group 3). Conditions like chronic obstructive pulmonary disease or sleep apnoea can lead to low oxygen, which causes the blood vessels to tighten. There is also chronic thromboembolic pulmonary hypertension (Group 4). This is caused by old blood clots blocking the arteries in the lungs, and it can be treated with surgery. Additionally, some patients may have PH with unclear causes (Group 5), which may be linked to blood disorders or other medical conditions. 

Here we are mainly talking about Group 1 PAH which is often mistaken for usual “high blood pressure" that many develop as they get older. PAH is very different. It is a rare and progressive condition which the pressure is high only in the lung’s blood vessels. When these blood vessels become narrow and stiff, the right side of the heart has to pump much harder. Over time, this leads to symptoms like shortness of breath, fatigue, and leg swelling. It is important to understand that regular blood pressure medications will not work for PAH. PAH requires its own special tests and treatment.

If a person has worsening shortness of breath, especially if they have risk factors such as an autoimmune disease like scleroderma or lupus, a history of liver disease, blood clots in the lungs, certain congenital heart conditions, or a family history of PAH, it is advisable to see a heart or lung specialist. Some hospitals also have specialised clinics for PH. The specialist will order the appropriate tests such as heart ultrasound (echocardiogram) to check pressure, right heart catheter to measure pressure more accurately, blood tests, CT scan, or sleep study to find the cause. Once diagnosis is confirmed, the medical team will create a personalised plan that is suitable for the patient.

Living Well with PAH

While there is currently no cure for Group 1 PAH, there are medications specially for PAH. These medications help relax and open the blood vessels in the lungs which reduces the pressure on the heart. For more complex or other groups of PH cases, doctors may recommend a therapeutic trial to assess how the patient responds and whether the medication is suitable for them. When starting these medications, patients may have side effects like headaches or flushing. These usually get better with time and can be managed effectively with support from the healthcare team. 

A simple and practical way of supporting patients is by adjusting their daily activities. Patients are encouraged to pace themselves by listening to their bodies, breaking tasks into smaller steps, and resting before exhaustion sets in. Supervised pulmonary rehabilitation can also help them build strength. Adopting a low-sodium diet helps prevent fluid retention, and having smaller, more frequent meals can ease breathlessness. It is essential to avoid smoking, hot tubs, and certain over-the-counter cold medicines. As infections can be more serious for those with PAH, staying up to date with vaccinations including the yearly flu shot, is important. Those who plan to travel, especially by air, should consult their doctor beforehand.

Apart from medication and daily habits, social support matters too. Many people experience anxiety after being diagnosed and that is normal. Joining support groups allows patients to connect with others who have similar conditions and to receive helpful guidance and encouragement. They can also learn more about PAH and be empowered to speak up for their needs.

A diagnosis of PAH can be life-changing, but it is not the end. With early diagnosis, the right treatment, good daily care, and strong support, many people with PAH continue to live full and active lives.