Overview
Diagnosis
Pulmonary hypertension is challenging to identify early because it rarely shows up during a routine physical exam. Even in more advanced stages, its symptoms resemble those of other heart and lung conditions. To diagnose the condition, a healthcare professional reviews your symptoms and asks questions about your medical and family history.
Tests to diagnose pulmonary hypertension may include:
• Blood tests to help find the cause and check for complications
• Chest X-ray to examine the heart, lungs and chest for related lung conditions
• Electrocardiogram to record the electrical activity of the heart and show how it beats
• Echocardiogram to show how blood flows through the heart and valves and to check treatment response
An echocardiogram may also be performed during exercise on a treadmill or stationary bike to see how activity affects the heart. You may wear a mask that measures how well the heart and lungs use oxygen and carbon dioxide.
Right heart catheterization may be used to confirm the diagnosis if an echocardiogram suggests pulmonary hypertension. A thin catheter is inserted into a blood vessel, usually in the neck, and guided into the right heart chamber and pulmonary artery to measure blood pressure.
Other tests may check the condition of the lungs and pulmonary arteries or determine the cause of pulmonary hypertension:
• Exercise stress tests to show how the heart reacts to physical activity
• CT scan to create detailed images of the body; contrast dye may be used to define blood vessels
• Cardiac CT scan to show the size of the heart and detect blockages in pulmonary arteries
• MRI to show blood flow in the pulmonary arteries and assess right heart function
• Lung function tests to measure lung capacity and airflow
• Sleep study to diagnose sleep apnea, a possible cause of pulmonary hypertension
• Ventilation/perfusion scan to examine airflow and blood flow using a tracer
• Lung biopsy, rarely used, to check for underlying causes
Genetic testing may be recommended to screen for gene changes linked to pulmonary hypertension. If certain gene changes are found, other family members may need screening.
Pulmonary hypertension functional classification helps describe how symptoms affect daily activities:
• Class I: No symptoms during rest or exercise
• Class II: No symptoms at rest; mild symptoms such as shortness of breath or mild chest pain during routine activities
• Class III: Comfortable at rest but symptoms occur with simple tasks such as dressing or meal preparation
• Class IV: Symptoms occur at rest and increase with any activity
Risk stratification tools that combine symptoms and test results help determine the most appropriate treatment.
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Cardiac catheterization
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Treatment
Although there is no cure for pulmonary hypertension, treatment can improve symptoms, slow disease progression and help you live longer. Finding the right approach may take time, and treatment is often complex, requiring regular medical checkups.
Medications may be prescribed to relieve symptoms and prevent complications. Treatment options include:
• Vasodilators to relax and widen blood vessels, improve blood flow and reduce pressure; they may be inhaled, taken orally or given through a vein
• Soluble guanylate cyclase stimulators to relax pulmonary arteries and lower lung pressure
• Endothelin receptor antagonists to reverse blood vessel narrowing and improve energy levels
• Phosphodiesterase 5 inhibitors to increase blood flow through the lungs
• High-dose calcium channel blockers to relax vessel walls, effective for a small number of people
• Blood thinners to prevent clot formation, with an increased risk of bleeding
• Digoxin to help the heart beat stronger and regulate rhythm
• Diuretics to remove excess fluid and reduce strain on the heart
• Oxygen therapy for those living at high altitudes, with sleep apnea or with low oxygen levels
If medicines do not adequately control symptoms, surgery or other procedures may be recommended:
Atrial septostomy may be considered when symptoms continue despite medicine. A small opening is created between the upper heart chambers to reduce pressure on the right side of the heart. Risks may include arrhythmias.
Lung or heart-lung transplant may be an option, particularly for younger individuals with idiopathic pulmonary arterial hypertension. After transplantation, lifelong medicines are needed to prevent organ rejection.
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