Pulmonary Hypertension: Elevated Blood Pressure in the Pulmonary Circulation

Pulmonary hypertension is a condition characterized by increased blood pressure in the pulmonary arteries. This phenomenon can develop for various reasons, and the diagnosis is typically made based on an echocardiogram. However, for an accurate diagnosis, specialized cardiac catheterization may also be used, though this is rarely necessary.

The increased pressure in the pulmonary arteries can be temporary, but in many cases, it can persist, leading to pulmonary hypertension. Often, an underlying disease affects the cardiovascular system, such as congenital or acquired abnormalities, or reduced compliance of the left ventricle. Treating these underlying conditions can improve the patient’s status.

The most common form of pulmonary hypertension is pulmonary arterial hypertension (PAH), the exact cause of which is not yet fully understood. The disease is progressive and can have severe consequences, making early diagnosis important. PAH affects a small portion of the population, most frequently occurring in younger women, but it can develop at any age. Currently, it is not curable, but it can be managed with medication.

Causes of Pulmonary Hypertension

Several factors can contribute to the development of pulmonary hypertension. High blood pressure in the pulmonary arteries is most often a consequence of conditions that obstruct circulation in the small blood circulation. These conditions can be temporary or permanent and may be associated with various diseases.

Cardiovascular disorders, such as congenital heart defects or cardiomyopathy, often play a role in the development of pulmonary hypertension. Reduced compliance of the left ventricle can also contribute to the pathological condition. Appropriate treatments, such as therapy for cardiovascular diseases, can lead to significant improvement in symptoms and measured outcomes.

Additionally, lung diseases, such as chronic obstructive pulmonary disease (COPD) or pulmonary fibrosis, can also contribute to disturbances in blood flow in the pulmonary arteries, leading to higher blood pressure in the small circulation. In the case of the primary form of pulmonary hypertension, pulmonary arterial hypertension, the exact causes are not always clear, but structural changes in the pulmonary arteries, such as narrowing, cause an increase in blood pressure.

Symptoms of Pulmonary Hypertension

In the early stages of pulmonary hypertension, symptoms are often nonspecific, making diagnosis difficult. Affected individuals often experience fatigue, reduced exercise tolerance, exertional dyspnea, chest pain, and chronic cough. Fatigue and shortness of breath are the most common complaints, but these can vary from person to person.

As the disease progresses, additional symptoms may appear, such as unexpected fainting, leg swelling, rapid heartbeat, and shortness of breath, which can occur even with minimal exertion. Increased abdominal girth may also occur due to heart failure. Diagnosis is typically made based on an echocardiogram, but more precise measurements may rarely require cardiac catheterization.

Treatment of Pulmonary Hypertension

The treatment of pulmonary hypertension varies depending on the underlying causes. If a treatable condition is responsible for the high pressure in the small circulation, addressing the underlying disease can lead to significant improvement. For example, treating heart or lung diseases can help alleviate symptoms.

In the case of pulmonary arterial hypertension, where there is no underlying disease, early treatment is crucial for slowing disease progression and improving quality of life. Various medication therapies, including combination treatments, are available to help prevent the condition from worsening and to extend patients’ lives.

In certain cases, such as chronic thromboembolic pulmonary hypertension (CTEPH), surgical intervention may also be necessary. In advanced stages, heart and lung transplantation may be considered, but these procedures are rarely feasible due to donor shortages or the patients’ conditions.

Treatment and care occur at appropriate specialized centers, such as PAH centers, where the latest therapies are applied. The Pulmonary Vascular Society assists those suffering from PAH in various areas of daily life, providing necessary support and information.