Pulmonary hypertension (PH) is a disease characterized by increased blood pressure in pulmonary circulation.
PH is classified in 5 major groups based on on the basis of similarities in pathophysiology, clinical presentation, and therapeutic options:
Group 1 - Pulmonary arterial hypertension (PAH)
Idiopathic, heritable, indused by drugs&toxins, associated with connective tissue disease, HIV, portal hypertension, congenital heart disease
Group 2. Pulmonary Hypertension (PH) caused by left- heart disease
Group 3. Pulmonary Hypertension (PH) due to lung disease and/or hypoxaemia
Group 4. Pulmonary hypertension (PH) due to pulmonary artery obstructions
Group 5. Pulmonary Hypertension (PH) miscellaneous (unclear or multifactorial
Group1, pulmonary arterial hypertension (PAH), is a progressive disease that slowly gets worse and can be life-threatening if left untreated. It is characterised by increased blood flow in the pulmonary arteries (vessels that carry blood from the heart to the lungs).Blood vessels contract and narrow and this leads to reduce blood flow to the lungs and in increase pulmonary artery pressure (PAP) and pulmonary vascular resistance (PVR).
As the disease gets worse patients feel shortness of breath (dyspnea) and fatigue and if the disease is left untreated can lead to heart failure (with high mortality)
The symptoms are unclear and unspecific. As the disease progresses, patients become symptomatic:
- Increased dyspnea in exercise (and/or at rest)
- Dizziness fainting spells (syncope)
- Racing pulse or heart palpitations
- Chest pain
- Swelling in the ankles and feet
- Bluish color to the lips and skin (cyanosis)
Early diagnosis is difficult and should be done in special PH centers. Symptoms (dyspnea, dizziness and fatigue) are not specific and can easily be confused with other, more common conditions, such as asthma, chronic obstructive pulmonary disease (COPD) or sleep apnoea.
The diagnostic test should include:
- Blood tests
- TRIPLEX Echo/Electrocardiogram (ECG)
- CT scan or computed tomography scan
- Ventilation-perfusion scan
- Right heart catheterisation
All the above should be performed in special PH centers during the initial diagnosis but also to monitor the patient's progress.
Patients with PAH should be referred to special PH centers due to the complexity and specific nature of the disease.
Treatment includes drugs that work in 3 different pathophysiological pathways: the c-AMP (prostanoid receptor) pathway, the endothelin pathway, and the c-GMP pathway.
Risk assessment often makes early combination therapy imperative in order to achieve clinical goals as soon as possible. The progression of the disease or the insufficient response to a treatment or even the initial severity of a patient’s condition, often requires simultaneously administration of drugs targeting in all of the above pathways.