Conceptual illustration of vascular remodeling in the arteries of the lung, used in the scientific dissemination of Pulmonary Arterial Hypertension by Ferrer.

Diseases

Pulmonary arterial hypertension

Pulmonary arterial hypertension (PAH) is a condition that causes narrowing of the blood vessels in the lungs, thereby increasing the pressure in the pulmonary arteries. This forces the right side of the heart to work harder than normal to push blood through these ducts and can eventually lead to right ventricular failure and premature death. It is estimated that in Europe it affects between 15 and 50 people per million inhabitants, and has a higher prevalence in women than in men¹.

A doctor in a white coat holds a patient's hand on the desk with both hands in a gesture of empathy and explanation.

Although in most cases its cause is unknown (idiopathic PAH), the disease may have a genetic origin (hereditary PAH) or be associated with other diseases or conditions. These causes include the use of certain medications, illegal drugs, or toxic substances (diet pills, cocaine, or amphetamines), heart problems present from birth, infections such as HIV, liver diseases such as cirrhosis or portal hypertension, and connective tissue-related diseases such as scleroderma or lupus erythematosus.

The symptoms most frequently related to PAH are those that usually occur in PH: shortness of breath, fatigue, intolerance to exertion, chest pain, possibility of ankle swelling, dizziness, and fainting. It can lead to respiratory failure and death.

Rear view of a man in a black T-shirt resting a hand on the wall as he walks alongside a woman in a brown sweater in a sunlit hallway.

Close-up of the torso of a health care professional in a white medical coat and a stethoscope around her neck as she takes notes in a medical history on a clipboard.

PAH can affect all aspects of daily life, not only for patients but also for their caregivers, with a major impact on their quality of life, as well as their emotional and social state. Although there is no cure for PAH, there are treatment options that can slow the progression of the disease. Likewise, studies indicate that an early diagnosis is associated with greater survival, as it allows the appropriate treatment to begin. Finally, it is essential to adopt healthy measures such as quitting smoking, staying active or following a healthy diet. Every case of PAH is different, and many variables can affect life expectancy.

Humbert M, Kovacs G, Hoeper MM, Badagliacca R, Berger RMF, Brida M, et al; ESC/ERS Scientific Document Group. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J. 2023 Jan 6; 61(1):2200879.