Pulmonary hypertension

The World Health Organization (WHO) describes five groups of pulmonary hypertension classified according to cause or related condition^1,2:

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

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

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

Pulmonary hypertension due to chronic obstruction of the pulmonary blood vessels: This includes PH caused by blood clots in the pulmonary arteries (chronic thromboembolic pulmonary hypertension) and other vascular obstructions.

Pulmonary hypertension with unclear or multifactorial mechanisms: This includes cases where several causes are involved or where the specific cause is unclear, such as certain blood disorders, metabolic disorders, or systemic diseases like sarcoidosis.

The diseases associated with PH are mainly characterized by shortness of breath, fatigue, and exercise intolerance, which often progress to respiratory failure and ultimately death. Other typical symptoms include chest pain, dizziness, fainting, palpitations, and cyanosis (bluish discoloration of the lips and fingers due to lack of oxygenation)¹.

The development of PH is almost invariably associated with worsening symptoms and increased mortality, regardless of the underlying disease¹. Although there is no cure for some types of PH, important advances in knowledge, therapy, and prognosis have changed the outlook of the disease and improved quality of life¹.

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.