Types of pulmonary hypertension

Pulmonary Hypertension – Types

There are two primary types of pulmonary hypertension:

• Primary (idiopathic) – This rare disease has an unknown origin, and in some cases, it may be hereditary, even passing down through generations. Both congenital and acquired primary pulmonary hypertension involve an increase in the mass of the right ventricle and atherosclerotic changes in the pulmonary artery. According to WHO recommendations (2008), this condition is termed "idiopathic pulmonary hypertension" (IPH). It progresses rapidly, affecting 5-6 individuals per million, leading to disability, and if untreated, premature death. Diagnosis of IPH relies on excluding other known and more common causes of elevated pressure in the pulmonary circulation.

• Secondary – This type develops in association with other diseases. It can be linked to connective tissue diseases (such as systemic scleroderma, systemic lupus erythematosus, vasculitis), HIV infection, congenital heart defects, post-thromboembolism in the pulmonary artery, and acquired heart defects (e.g., mitral stenosis, aortic defects) in diseases with insufficient left ventricle function. Pulmonary hypertension may accompany lung diseases, but it typically does not reach a high degree. Over a hundred diseases are known to lead to secondary pulmonary hypertension, where it acts as a complication of the underlying disease.

Based on the degree of pressure increase in the pulmonary artery (determined by cardiac catheterization or echocardiography), three degrees are recognized:
First degree – 25-45 mm Hg.
Second degree - 45-65 mm Hg.
Third degree - more than 65 mm Hg.

Clinical severity of circulatory failure categorizes pulmonary hypertension into four classes:

• Class I: Patients with pulmonary hypertension without impairments in physical activity. Normal exercise does not cause discomfort.

• Class II: Patients with pulmonary hypertension experiencing minor impairment during physical activity. Rest is comfortable, but routine physical activity results in dizziness, shortness of breath, chest pain, and weakness.

• Class III: Patients with significant impairment of physical activity due to pulmonary hypertension. Even minor physical activity leads to dizziness, shortness of breath, chest pain, and weakness.

• Class IV: Patients with severe pulmonary hypertension, experiencing intense dizziness, shortness of breath, chest pain, weakness with minimal exertion, and even at rest.

The prognosis for pulmonary hypertension patients depends on its underlying cause. Some diseases can be successfully treated, while idiopathic pulmonary arterial hypertension is progressive, with symptoms worsening over time. Ongoing global research aims to unravel the subtle mechanisms of this disease, leading to the development of new treatment methods. Many medications widely used in Western countries are registered in Ukraine, and it is anticipated that even more effective drugs will be introduced for treatment in the future.

It is crucial to promptly diagnose the disease and select the optimal treatment regimen for patients.