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Pulmonary arterial hypertension

Hipertensió arterial pulmonar a Vall d'Hebron

Pulmonary arterial hypertension (PAH) is a disorder of the arteries that connect the lungs to the heart. Symptoms are shortness of breath or laboured breathing (dyspnoea). 


Pulmonary arterial hypertension is a rare and serious condition that affects the pulmonary artery system. As the disease progresses, blood flow reduces. To compensate for this, the right side of the heart grows excessively, creating breathing difficulties.

It is defined by a rise in pulmonary artery pressure caused by abnormalities in the precapillary pulmonary arterioles due to uncontrolled hypertrophy, hyperplasia and proliferation.



Dyspnoea, syncope, palpitations.


Who does it affect?

It affects children and adults, especially women (65-80%) and usually appears in adulthood.

In around 50% of cases of pulmonary arterial hypertension the cause is unknown. The other 50% are related to:

  • Taking drugs and toxic substances, such as some anorectics or colza oil.
  • Autoimmune disorders such as systemic sclerosis
  • Congenital heart disease
  • HIV infection
  • Liver disease
  • Rarer causes

In Spain there are 16 cases for every million adults and the incidence rate is 3.7 for every million adults per year.


How is it diagnosed?

Diagnosis is via a series of tests:

  • Full lung function test
  • Lung scintigraphy
  • Blood test
  • Echocardiogram
  • Right heart catheterization  


What is the treatment and how effective is it?  Can it be cured?

Although they will not cure it, there are several treatments that can significantly improve the condition, its prognosis and patients’ quality of life.

We are fortunate to have access to all the pharmaceuticals beneficial in fighting this disease:  

  • Endothelin receptor antagonists (macitentan, bosentan, ambrisentan)
  • Phosphodiesterase-5 inhibitors (tadalafil, sildenafil)
  • Prostacyclin analogues (epoprostenol, treprostinil and iloprost)
  • Direct simulators of soluble guanylate cyclase (riociguat)  
  • Prostacyclin receptor Agonists (selexipag)

The decision about the most suitable drugs for each patient needs to be made by centres with experience. Lastly, if these measures are insufficient, a lung transplant can be considered in some cases.


How can I prevent this disease?

Taking anorectics, amphetamines and cocaine should be avoided. The HIV virus can also cause this condition and all factors, principally alcohol, that may lead to liver failure.  Apart from these measures there are no other means of prevention and efforts should focus on early detection.

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Dr. Jaume Joan
Ferrer Sancho
Head of Department
Sra. Ana Elena
Ruiz Alcaraz
Nursing Supervisor
Internal Medicine
Sra. Julia
Salvador Lodosa
Nursing Supervisor
Internal Medicine
Sra. Natalia
González Otero
Dr. Maria Luiza
De Souza Galvão
Medical Coordinator
Dra. Ana Belén
Méndez Fernández
Medical Coordinator
Dra. Laura
Dos Subirà
Head of Section
Lead Researcher
Cardiovascular Diseases
Dr. Ignacio
Ferreira González
Head of Unit
Lead Researcher
Cardiovascular Diseases
Dra. Begoña
Benito Villabriga
Dr. Jaume
Francisco Pascual
Dr. Alfredo
Guillén del Castillo
Dra. Asunción
Torrents Fernández
Dra. Laura
Galian Gay