Pulmonary hypertension, or PH, is a form of high blood pressure that specifically affects the blood vessels connecting the heart and lungs. Unlike systemic hypertension, which impacts arteries throughout the body, PH is characterized by elevated pressure within the pulmonary arteries. The heart’s right side pumps blood through the lungs to receive oxygen, a task that normally occurs under low pressure.
In PH, these arteries become narrow or damaged, forcing the right side of the heart to work harder to circulate blood, which can weaken the heart muscle over time. A diagnosis is considered when the average pulmonary artery pressure exceeds 20 mm Hg at rest, compared to the normal range of 11 to 20 mm Hg.
Symptoms and Physical Manifestations
The initial symptoms of pulmonary hypertension can be subtle, developing slowly over months or years. The earliest sign is often shortness of breath, which first appears during physical activity and may later occur at rest. As the condition progresses and puts more strain on the heart, other symptoms emerge.
These include chronic fatigue, dizziness or fainting spells, chest pressure, and a racing heartbeat. Swelling (edema) can develop in the ankles, legs, and abdomen because the overworked right side of the heart struggles to move fluid effectively. A bluish tint on the lips or skin, called cyanosis, may also appear, indicating low oxygen levels.
Underlying Causes and Classification
Pulmonary hypertension can arise from numerous underlying issues. To clarify the origins, the World Health Organization (WHO) categorizes PH into five groups based on its cause, which helps guide treatment. One category has no identifiable cause (idiopathic) or is linked to genetic factors.
Another group is associated with conditions affecting the left side of the heart, where inefficient pumping causes blood to back up into the lungs. Lung diseases like chronic obstructive pulmonary disease (COPD) or conditions causing low oxygen levels can also lead to PH. Other cases are connected to diseases like connective tissue disorders, congenital heart defects, or liver disease. A distinct form, chronic thromboembolic pulmonary hypertension (CTEPH), results from blood clots that block or scar the pulmonary arteries.
The Diagnostic Process
Diagnosing pulmonary hypertension involves tests to confirm high pressure in the pulmonary arteries and find its cause. A physician will start with a physical exam, listening to the heart and lungs and checking for signs like swelling in the legs, and will also review your medical history. An echocardiogram, a non-invasive ultrasound of the heart, is a primary screening tool that can estimate pulmonary artery pressure and assess the right heart’s function.
While an echocardiogram strongly suggests PH, a right heart catheterization is the definitive test. This procedure involves guiding a thin tube into the heart and pulmonary artery to measure blood pressure directly. Other tests may be used to gather more information. A chest X-ray can show if the heart or pulmonary arteries are enlarged, while CT scans provide detailed images to identify issues like blood clots.
Medical Management and Therapies
While there is no cure for pulmonary hypertension, treatments can manage symptoms, slow its progression, and improve quality of life. Plans are individualized based on the type and severity of the PH. Medications are a primary component of management.
- Vasodilators work to open and relax narrowed blood vessels and can be administered orally, inhaled, or via an intravenous (IV) pump.
- Endothelin receptor antagonists help prevent blood vessels from constricting.
- Anticoagulants (blood thinners) are used for PH caused by chronic blood clots to prevent new ones from forming.
- Diuretics, or “water pills,” help reduce fluid buildup in the body.
Supportive therapies include oxygen therapy to ease shortness of breath for those with low blood oxygen levels. In specific, advanced cases that do not respond to other treatments, surgical procedures like a lung transplant may be an option.
Living with Pulmonary Hypertension
Managing pulmonary hypertension is a long-term commitment involving ongoing medical care and lifestyle adjustments. Regular follow-up appointments are necessary to monitor the condition and adapt treatments. Lifestyle modifications play a large part in daily management.
Doctor-approved exercise programs, like pulmonary rehabilitation, can improve physical conditioning, while a low-sodium diet helps manage fluid retention. It is also beneficial to avoid triggers that can worsen symptoms, such as high altitudes and smoking.
Living with a chronic illness also means learning to conserve energy and adapt daily routines. Simple changes, like rearranging a kitchen to keep frequently used items within easy reach, can make a difference. Many people find it helpful to connect with others through support groups to share experiences and coping strategies.