Understanding Pulmonary Hypertension (PH or PHTN): Causes, Symptoms, and Management

What is it?

Pulmonary hypertension is a type of high blood pressure that affects the arteries in the lungs and the right side of the heart.
In one form of pulmonary hypertension, called pulmonary arterial hypertension (PAH), blood vessels in the lungs are narrowed, blocked or destroyed. The damage slows blood flow through the lungs, and blood pressure in the lung arteries rises. The heart must work harder to pump blood through the lungs. The extra effort eventually causes the heart muscle to become weak and fail.
In some people, pulmonary hypertension slowly gets worse and can be life-threatening. Although there's no cure for some types of pulmonary hypertension, treatment can help reduce symptoms and improve quality of life.

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Additional names

This group contains additional names:
- Pulmonary veno-occlusive disease
- Right heart failure due to pulmonary hypertension
- Thromboembolic pulmonary hypertension

Signs & symptoms

Signs and symptoms of pulmonary hypertension develop slowly, over months or even years. Symptoms get worse as the disease progresses.

Symptoms may include:
- Shortness of breath (dyspnea), initially while exercising and eventually while at rest
- Fatigue
- Dizziness or fainting spells (syncope)
- Chest pressure or pain
- Swelling (edema) in ankles, legs and eventually in your abdomen (ascites)
- Bluish color in lips and skin (cyanosis)
- Racing pulse or heart palpitations

Diagnosis

Pulmonary hypertension is hard to diagnose early because it's not often detected in a routine physical exam. Even when the condition is more advanced, its signs and symptoms are similar to those of other heart and lung conditions.
Diagnosis includes a physical exam, medical anamnesis about signs and symptoms and tests. Tests for pulmonary hypertension may include:
- Blood tests
- Chest X-ray: To get pictures of the heart, lungs and chest. It can show enlargement of the right ventricle of the heart or the pulmonary arteries, which can occur in pulmonary hypertension.
- Electrocardiogram (ECG): Noninvasive test that shows heart's electrical patterns and can detect abnormal heartbeats.
- Echocardiogram
- Right heart catheterization: If an echocardiogram reveals pulmonary hypertension, right heart catheterization will be performed to confirm the diagnosis.

Treatment

There's no cure for pulmonary hypertension, but there are treatments that helpt to manage the condition. Treatment may help improve 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.
When pulmonary hypertension is caused by another condition,there is a need to treat the underlying cause whenever possible.

Medications:
- Blood vessel dilators (vasodilators): To relax and open narrowed blood vessels, improving blood flow.
- Guanylate cyclase (GSC) stimulator: Riociguat (Adempas) increases nitric oxide in the body, which relaxes the pulmonary arteries and lowers pressure within them
- Endothelin receptor antagonists: These medications reverse the effect of endothelin, a substance in the walls of blood vessels that causes them to narrow.
- Sildenafil and tadalafil: They open the blood vessels in the lungs and allow blood to flow through more easily.
- High-dose calcium channel blockers: These drugs help relax the muscles in the walls of the blood vessels.
- Warfarin: Warfarin is a type of drug called an anticoagulant (blood thinner). They are used to help prevent blood clots in the lung's arteries.
- Digoxin: Digoxin (Lanoxin) helps the heartbeat stronger and pump more blood.
- Diuretics: Commonly known as water pills, these medications help the kidneys remove excess fluid from the body. This reduces the amount of work the heart has to do. They may also be used to limit fluid buildup in the lungs, legs and abdomen.
- Oxygen therapy

Surgery
- Atrial septostomy: If medications don't control pulmonary hypertension, this open-heart surgery might be an option. In an atrial septostomy, a surgeon creates an opening between the upper left and right chambers of your heart (atria) to relieve the pressure on the right side of the heart.
- Transplantation: In some cases, a lung or heart-lung transplant might be an option, especially for younger people who have idiopathic pulmonary arterial hypertension.

☝️ This is not a substitute for professional medical advice. Please consult with your physician before making any medical decision.

Learn more about our editorial process for content accuracy.

Source of Information - We analyze big data of more than 23 million patients de-identified medical records from the following sources:

National Institution of Health ∙ World Health Organization ∙ University of Illinois hospital