Pulmonary hypertension

The World Health Organization (WHO) describes five groups of pulmonary hypertension classified by cause or related condition:

Pulmonary arterial hypertension (PAH): This group includes diseases that are characterized by narrowing of the small pulmonary arteries, such as idiopathic PAH, hereditary PAH, and that caused by certain medications, infections, connective tissue diseases, or autoimmune diseases.

Pulmonary hypertension due to diseases of the left heart: This lists cases in which PH is caused by problems on the left side of the heart, such as heart failure or heart valve disease.

Pulmonary hypertension due to lung diseases: This group includes PH caused by lung diseases such as chronic obstructive pulmonary disease (COPD), interstitial lung diseases (ILD), and others.

Pulmonary hypertension due to chronic blockage in the blood vessels of the lungs: This is where PH caused by blood clots in the pulmonary arteries (chronic pulmonary thromboembolism) and other vascular obstructions comes in.

Pulmonary hypertension of unclear causes or multiple factors: This group includes cases where there are several causes involved or where the specific cause is unclear, such as in certain blood, metabolic disorders, or systemic disorders such as sarcoidosis.

The different pathologies associated with PH are characterized, above all, by shortness of breath, fatigue and intolerance to exertion, which usually evolve into respiratory failure and, finally, lead to death. Other typical symptoms can be chest pain, dizziness, fainting, palpitations and cyanosis (bluish coloration of the lips and fingers, due to lack of oxygenation).

The development of PH is almost invariably associated with worsening symptoms and increased mortality, regardless of the underlying disease. Although there is no cure for some types of PH, important steps have been taken in terms of knowledge, therapy, and prognosis that have changed the perspective of the disease and improved quality of life.

1. 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.

2. Hoeper MM, Humbert M, Souza R, Idrees M, Kawut SM, Sliwa-Hahnle K, et al. A global view of pulmonary hypertension. Lancet Respir Med. 2016 Apr; 4(4):306-22.