NOP 19/0447f(1)a April 2019
This article was developed in collaboration with Actelion Pharmaceuticals UK who also provided funding.
Contributors to this article did not receive payment for their involvement.
Prof David Kiely
Director, Sheffield Pulmonary Vascular Disease Unit
Diagnosis for pulmonary arterial hypertension (PAH) takes too long and the condition can often be misdiagnosed. We need to take another approach says a leading specialist.
“It’s vital that diagnosis is improved for patients with pulmonary arterial hypertension (PAH). It is a chronic and incurable heart and lung condition that affects some 3,000 people in the UK,” says Professor David Kiely, Director of the Sheffield Pulmonary Vascular Disease Unit.
“Multiple new PAH treatments are available, which have dramatically improved survival rates,” he says. “Early diagnosis means greater opportunity to access these treatments, meaning better outcomes for patients.
“Unfortunately, diagnosis of PAH can take about two years and many people remain undiagnosed — a situation that is common in many other countries around the world and hasn’t changed significantly over the last two to three decades.”
Average survival is just 2-3 years if left untreated
PAH — a rare form of pulmonary hypertension (PH) — narrows the small blood vessels that lead to the lungs, causing patients to struggle for breath. In some types, such as idiopathic PAH, average survival is just two to three years if left untreated.
The trouble is, breathlessness — the main symptom of PAH — also presents in many other common conditions, which means it’s easy for patients to be misdiagnosed. “Our current approach to investigating breathlessness doesn’t help identify rare conditions,” explains Professor Kiely. “So, we need to apply new and alternative approaches, including the use of artificial intelligence techniques that have the potential to identify those at greater risk of idiopathic PAH.”
Importance of referral to specialist care
Specialist management is essential for patients with PAH. When the condition is suspected it’s incredibly important that adult patients are referred to one of seven specialist pulmonary hypertension centres around the UK, with children referred to a specialist centre in London.
“These centres allow patients to receive a speedy diagnosis and begin – often complex – drug therapies,” says Professor Kiely. “These centres have been big success stories and greatly increased the number of people receiving treatment.”
“Once patients have started treatment, it’s important they are regularly monitored and assessed with a variety of different, usually non-invasive tests. That’s why it’s important patients are assessed in specialist centres where highly skilled, multi-professional teams are experienced in managing this challenging condition.”