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 the blood vessels in the lungs to narrow, increasing pressure in the pulmonary arteries. As a result, the right side of the heart must work harder than normal to pump blood through these narrowed vessels. Over time, this can cause right ventricular failure and premature death¹. It is estimated that in Europe PAH affects between 15 and 50 people per million inhabitants, with a higher prevalence in women than in men².

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 the cause is unknown (idiopathic PAH) in most cases, the disease can be genetic (hereditary PAH) or associated with other diseases or conditions. These latter cases include the use of certain medications, illegal drugs or toxins (diet pills, cocaine, or amphetamines), heart defects present from birth, infections such as HIV, liver diseases such as cirrhosis or portal hypertension, and certain connective tissue disorders like scleroderma or lupus.

The most common symptoms of PAH are those typically seen in PH: shortness of breath, fatigue exercise intolerance, chest pain, possible ankle swelling, dizziness and fainting. These symptoms may lead to respiratory failure and death³.

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, causing a major impact on quality of life as well as emotional and social well-being. Although there is no cure for PAH, treatment options exist that can slow disease progression. Studies indicate that early diagnosis is associated with improved survival, as it allows appropriate treatment to begin earlier⁴. It is also essential to adopt healthy lifestyle measures such as quitting smoking, staying active, and maintaining a healthy diet. Each PAH case is different, and many variables may affect life expectancy.

Hassoun PM. Pulmonary Arterial Hypertension. N Engl J Med. 2021;385:2361-2376.
Humbert M. The burden of pulmonary hypertension Eur Respir J. 2007;30:1-2.
Humbert M, Kovacs G, Hoeper MM, Badagliacca R, Berger RMF, Brida M, et al. 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2022;43(38):3618-3731.
Humbert M, Sitbon O, Chaouat A, et al. Survival in patients with idiopathic, familial, and anorexigen-associated pulmonary arterial hypertension in the modern management era. Circulation 2010;122:156–163.