Clinical Services Department MC-21
Pulmonary hypertension is a type of high blood pressure that affects the arteries in your lungs and the right side of your heart. Blood vessels in your lungs are narrowed, blocked or destroyed. The damage slows blood flow through your lungs, and blood pressure in the lung arteries rises. Your heart must work harder to pump blood through your lungs. The extra effort eventually causes your heart muscle to become weak and fail.
The diagnosis is often delayed because the presenting features are frequently attributed incorrectly to age, deconditioning, or a coexisting or alternate medical condition. As a result, pulmonary hypertension is often not suspected until symptoms become severe or serious.
Pulmonary hypertension symptoms include:
• Shortness of breath (dyspnea), initially while exercising and eventually while at rest
• Dizziness or fainting spells (syncope)
• Chest pressure or pain
• Swelling (edema) in your ankles, legs and eventually in your abdomen (ascites)
• Bluish color to your lips and skin (cyanosis)
• Racing pulse or heart palpitations
Once you’ve been diagnosed with pulmonary hypertension, your doctor might classify the severity of your disease into one of several classes, including:
• Class I: Although you’ve been diagnosed with pulmonary hypertension, you have no symptoms with normal activity.
• Class II: You don’t have symptoms at rest, but you have symptoms such as fatigue, shortness of breath or chest pain with normal activity.
• Class III: You’re comfortable at rest, but have symptoms when you’re physically active.
• Class IV: You have symptoms while at rest and during physical activity.
There’s no cure for pulmonary hypertension, but your doctors can prescribe treatments to help you manage your condition. Treatment may help improve your symptoms and slow the progress of pulmonary hypertension. It often takes some time to find the most appropriate treatment for pulmonary hypertension. The treatments are often complex and require extensive follow-up care.
• Blood vessel dilators (vasodilators): vasodilators relax and open narrowed blood vessels, improving blood flow. One of the most commonly prescribed vasodilators for pulmonary hypertension is epoprostenol (Flolan, Veletri).
• Guanylate cyclase (GSC) stimulators: Riociguat (Adempas) increases nitric oxide in the body, which relaxes the pulmonary arteries and lowers pressure within them. Side effects include nausea, dizziness and fainting. You should not take GSC stimulators if you’re pregnant.
• Endothelin receptor antagonists: These medications reverse the effect of endothelin, a substance in the walls of blood vessels that causes them to narrow. Such drugs include bosentan (Tracleer), macitentan (Opsumit), and ambrisentan (Letairis). These drugs may improve your energy level and symptoms. However, they can damage your liver. You may need monthly blood tests to check your liver function. Endothelin receptor antagonists shouldn’t be taken if you’re pregnant.
• Sildenafil and tadalafil: Sildenafil (Revatio, Viagra) and tadalafil (Adcirca, Cialis) are commonly used to treat erectile dysfunction, but they also open the blood vessels in the lungs and allow blood to flow through more easily. Side effects can include an upset stomach, headache and vision problems.
• High-dose calcium channel blockers: these drugs help relax the muscles in the walls of your blood vessels. They include amlodipine (Norvasc), diltiazem (Cardizem, Tiazac, others) and nifedipine (Procardia, others). Although calcium channel blockers can be effective, only a small number of people with pulmonary hypertension improve while taking them.
Although medical treatment can’t cure pulmonary hypertension, it can lessen symptoms. Lifestyle changes also can help improve your condition. Consider these tips:
• Get plenty of rest
• Stay as active as possible
• Don’t smoke
• Don’t travel to or live at high altitudes
• Eat healthy and manage your weight
• Ask your doctor about medications
• Get regular doctor checkups
• Get recommended vaccines
• Cleveland Clinic: https://my.clevelandclinic.org/health/diseases/6530-pulmonary-hypertension-ph/management-and-treatment
• Mayo Clinic: https://www.mayoclinic.org/diseases-conditions/pulmonary-hypertension/symptoms-causes/syc-20350697