“Different clinical and pathological features characterise the diverse clinical pulmonary hypertension (PH) groups”1 The following, most up-to-date version of the classification is as follows:

Pulmonary arterial hypertension (PAH) is a progressive disease that reduces blood flow and increases pressure in the arteries of the lungs.

Chronic thromboembolic pulmonary hypertension (CTEPH), similarly to other types of pulmonary hypertension (PH), reduces blood flow and increases pressure in the blood vessels of the lung1. Breaking its name down helps us to understand what CTEPH is.

People with Pulmonary Hypertension experience a range of symptoms that vary in intensity depending on the severity of their condition, however the most common symptom is

Pulmonary arterial hypertension is often not diagnosed in a timely manner because its early symptoms can be confused with those of other cardio-respiratory conditions. Symptoms can initially be subtle and relatively non-specific. A process of exclusion generally makes diagnosis of PAH by looking for and ruling out other diseases (see below).

• Basic treatment a) Oral anticoagulation (pills for thinning the blood) b) Diuretics (pills for removing water) c) Oxygen treatment

In the past 15-20 years enormous progress has been made in the treatment of pulmonary arterial hypertension, one of the five subtypes of pulmonary hypertension (WHO group 1), with nine drugs approved by the EU regulatory authorities.

Medical practice guidelines are peer reviewed recommendations developed by task forces nominated by Scientific Societies and based on available evidence and expert opinion.

In the past 15-20 years enormous progress has been made in the treatment of pulmonary arterial hypertension, PAH, one of the five subtypes of pulmonary hypertension (WHO group 1). The new treatments have led to significant improvements for patients in terms of symptoms, disease progression and survival.

Here are some common abbreviations. They are taken from the 2022 ERS/ESC PH guidelines.

Complete and extensive diagnostic workup is mandatory in children with suspected pulmonary hypertension and, even if somewhat similar to that of an adult, must be adapted to the age of the patient.

Pregnancy poses a risk for women with any type of pulmonary hypertension (PH) as it is associated with high rates of maternal illness and even death.1 For this reason, women of childbearing age with PH are recommended to use reliable contraceptive methods to avoid pregnancy, or to consider an early termination if they do get pregnant.2