About PH

What is PAH?

Pulmonary arterial hypertension (PAH) is a progressive disease that reduces blood flow and increases pressure in the arteries of the lungs. What causes PAH? The pulmonary arteries are the large blood vessels responsible for transporting blood from the heart to the lungs to pick up oxygen. In PAH, the cells lining the inside of these […]

What is CTEPH?

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.

Symptoms

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 breathlessness: shortness of breath (dyspnoea) with no obvious cause during exertion or even at rest.

Diagnosis

Diagnosis 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).

Treatment

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

Therapies

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.

ESC/ERS Guidelines

Medical practice guidelines are peer reviewed recommendations developed by task forces nominated by Scientific Societies and based on available evidence and expert opinion. An evaluation study revealed that “the acknowledgement of the value of the PH guidelines is uniformly high”.1

Research

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. In 2013 a soluble guanylate cyclase stimulator was […]

PH in children

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. The most frequent etiologies include congenital heart disease and idiopathic/heritable pulmonary hypertension but some pediatric specificties such as persistent pulmonary hypertension of the […]