Pulmonary hypertension is a hidden killer

Lisa Salmon reports on a difficult-to-diagnose disease.

IT’S easy to dismiss a condition no one really talks about as no big deal. But in the case of pulmonary hypertension (PH), it really is a big deal — so big, in fact, that if left untreated, the condition can kill within two years.

Hypertension means high blood pressure, and pulmonary hypertension occurs when the arteries in the lungs narrow and thicken, slowing the flow of blood through the pulmonary arteries to the lungs.

As a result, pressure in the arteries rises as the heart works harder to force the blood through. This can eventually lead to heart failure, when the heart becomes too weak to pump enough blood to the lungs.

The condition can affect people of any age, including children, but it’s three times more common in females — although it’s not known why — and symptoms typically start to present around 45-55 years of age.

Natalie Cole in 2008

Grammy-winning singer and actress Natalie Cole died from heart failure last New Year’s Eve aged 65, after struggling with pulmonary hypertension for six years.

Cole had idiopathic pulmonary hypertension, the form of the illness unrelated to other contributory diseases.

In a third of cases, the condition develops spontaneously like this, and it’s not known what the triggers are.

The rest of the time, for two-thirds of cases, PH is related to another condition, such as congenital heart disease, or connective tissue disorders and other autoimmune conditions like rheumatoid arthritis — though it’s important to note that lots of people with these conditions do not develop PH.

Having a family member with pulmonary hypertension also increases the risk of developing the disease.

PH can develop slowly, with early symptoms easily dismissed: shortness of breath during routine activity, fatigue, chest pain, a racing heartbeat, pain in the upper-right abdomen, and a decreased appetite.

The slow and vague onset of symptoms — alongside the general lack of awareness — means people with PH often face a significant delay in their diagnosis.

British research has found 47% of patients saw four or more doctors before being correctly diagnosed.

Plus, more than half (53%) waited at least a year, and more than a third (34%) waited more than two years before finding out what was actually wrong.

Sometimes, delayed diagnosis can also be because people are initially thought to have other, more common, conditions, such as asthma or another heart condition.

There’s currently no cure for PH — however, the prognosis for patients has improved considerably in recent years.

This is partly because four new drug therapies have been developed, that can relax the blood vessels in the lungs.

“You see varying responses, but with treatment, the average patient with PH lives seven or eight years — although some can live a lot longer,” says nurse consultant Iain Armstrong.

“That’s devastating and it’s still unfortunately an early death, but treatment can improve symptoms and make patients less breathless, and sometimes able to go back to work part-time, although rarely does treatment take away all symptoms.

“Its progression is unpredictable and people with pulmonary hypertension have to live with real uncertainty,” he adds.

“But with well-managed care and support, they can lead good, fulfilling lives, and that’s an important message.”