World Day

Pulmonary arterial hypertension: why listening to the breath improves diagnosis and treatment

From the new communication campaign in music conceived by Maestro Melozzi to the progress of new-generation drugs that directly intervene on biological mechanisms: the point on the rare disease that in Italia affects about 3,000 people, mostly women

by Roberto Badagliacca *

3' min read

Translated by AI
Versione italiana

3' min read

Translated by AI
Versione italiana

Breathing is the most natural act we perform, often without realising it. Only when we miss it do we realise how essential it is. For people living with pulmonary arterial hypertension (PAH), breathing is never an act to be taken for granted: it is a daily challenge that affects even the simplest gestures and affects the relationships and quality of life of those who suffer from it.

The Campaign in Music

On the occasion of World Pulmonary Arterial Hypertension Day on 5 May, returning to the subject of this rare disease is essential to increase public awareness and attention. Awareness-raising campaigns such as 'Aria di vita - and it's music for people with pulmonary arterial hypertension', promoted by MSD Italia, with the patronage of the patient associations Amip and Aipi, also help to bring the experience of people living with this disease back to the centre. The campaign, through the powerful and universal language of music, wanted to turn breath into notes. The stories and emotions of people suffering from this disease become musical notes of an unpublished and exclusive composition by Maestro Enrico Melozzi.

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The disease

Pulmonary arterial hypertension is a rare, progressive and severely disabling disease, characterised by increased pressure in the pulmonary arteries with a significant overload on the right heart: in Italia, an estimated 3,000 people, mostly women, live with this condition.

 

One of the most critical issues in the management of Iap is the diagnostic delay. The initial symptoms - dyspnoea and fatigue - are non-specific and can be confused with those of other pulmonary or cardiac diseases. It is not uncommon for up to two years to elapse from the onset of symptoms before a correct diagnosis is reached. When this happens, pulmonary vascular remodelling and functional heart damage may already be advanced, making the course of treatment more complex.

 

This is why early diagnosis is decisive. Recognising the disease early and directing patients to specialist referral centres enables multidisciplinary care and access to the best available options.

 

Research and therapy

In recent years, research has profoundly changed the natural history of Iap. Whereas in the past, in the most severe cases, the only prospect was lung transplantation, we now have numerous treatments that can improve symptoms, increase functional capacity, slow down disease progression and prolong survival.

 

Many currently used therapies act as vasodilators, improving blood flow in the pulmonary circulation and reducing the overload on the right heart. But the real paradigm shift comes from next-generation drugs, which act directly on the biological mechanisms responsible for the disease. In particular, some innovative therapies mainly interfere with the cell proliferation processes that fuel obstructive vascular remodelling, underlying the increase in pulmonary resistance and pulmonary arterial pressure.

 

This approach opens up new scenarios: not only symptom control, but concrete possibilities of affecting the evolution of the pathology. Scientific evidence shows a significant reduction in pulmonary vascular resistance, an improvement in exercise capacity and a favourable impact on patients' morbidity and mortality.

 

However, it remains very important to spread awareness and to encourage faster diagnostic pathways that allow patients to be referred to centres of reference to ensure timely treatment and the best treatment option for each individual.

 

Listening to the breath of those living with pulmonary arterial hypertension means recognising an often invisible fragility. But it also means grasping the signal of an evolving science that today, more than ever, can offer new perspectives on life.

* Head of Cardiac Intensive Care Unit, Department of Medical and Cardiovascular Sciences, Sapienza University/Policlinico Umberto I, Rome

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