Pulmonary Hypertension Care

UC San Diego Health is a global leader in the diagnosis, treatment and research of pulmonary hypertension.

We specialize in all types of pulmonary hypertension, including pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH). Our comprehensive CTEPH program receives referrals from around the globe and treats more patients than any other center in the world. We offer all types of available medical and surgical treatments, including pulmonary thromboendarterectomy (PTE) surgery, balloon pulmonary angioplasty (BPA), and pulmonary hypertension medical therapy.

What Is Pulmonary Hypertension?

Pulmonary hypertension (PH) is high pressure in the blood vessels that carry blood into the lungs. This can strain the arteries in the lungs and the right side of the heart and lead to serious problems.

Pulmonary hypertension can get worse with time, so it's important to accurately identify the cause as soon as possible. Newly diagnosed patients need a pulmonary hypertension specialist who can develop a customized treatment plan and monitor the response.

Pulmonary Hypertension Symptoms

Symptoms of pulmonary hypertension may come on suddenly or occur slowly over time. Signs of pulmonary hypertension can include:

• Shortness of breath
• Tiring quickly, especially when active
• Chest pain or pressure
• Fast heartbeat
• Swelling in the legs or ankles
• Fainting or dizzy spells
• Blue lips or fingernails (signs of low oxygen)
• Pain in the upper right side of the abdomen

These signs of pulmonary hypertension may be misdiagnosed as another condition. If you suspect you may have pulmonary hypertension, schedule an evaluation with our team of experts.

Causes and Types of Pulmonary Hypertension

Pulmonary hypertension is most often caused by another health condition, known as secondary PH. In many cases, treating this underlying condition can help prevent or manage pulmonary hypertension. Some of the most common causes of pulmonary hypertension in adults are:

• Left-sided heart or valvular disease
• Chronic lung conditions, such as chronic obstructive pulmonary disease (COPD), advanced bronchitis, cystic fibrosis or pulmonary fibrosis
• Blood clots in the lungs
• Autoimmune disease and genetic conditions, such as scleroderma, lupus or sickle cell disease
• Liver disease
• Sleep apnea and other breathing disorders
• Side effects of some drugs (such as diet pills or methamphetamine)

WHO Groups

A WHO group refers to the five broad categories of pulmonary hypertension as defined by the World Health Organization (WHO). These groups are based on the underlying cause of pulmonary hypertension:

• Group 1 – Pulmonary arterial hypertension (PAH): This group includes PH where the pulmonary arteries in the lungs become narrowed and thickened.
• Group 2 – Left-sided heart disease: Encompasses PH resulting from problems with the left side of the heart, such as left ventricular systolic dysfunction.
• Group 3 – Lung diseases and/or hypoxia: Includes PH caused by lung conditions like chronic obstructive pulmonary disease (COPD), or conditions that lead to low blood oxygen levels.
• Group 4 – Chronic thromboembolic pulmonary hypertension (CTEPH): Refers to PH caused by old blood clots in the pulmonary arteries.
• Group 5 – PH with unclear and/or multifactorial mechanisms: Includes PH caused by a variety of factors that are not easily categorized into the other four groups, such as blood disorders or metabolic problems.

Pulmonary Hypertension Diagnosis

Diagnosing pulmonary hypertension can be difficult because many of its signs and symptoms are similar to other heart or lung diseases, like coronary artery disease, asthma and COPD.

In many cases, our specialists at UC San Diego Health correct a previous diagnosis, finding one form of pulmonary hypertension when another has been assumed or when the patient has been living with a misdiagnosis. A correct diagnosis is critical for successful outcomes.

The following tests may be used to evaluate the cause of pulmonary hypertension:

• Echocardiogram
• V/Q scan
• CT scan
• Right-heart catheterization
• Pulmonary angiogram
• Blood tests
• Pulmonary function test
• MRI
• Cardiopulmonary exercise stress test

Pulmonary Hypertension Treatment Options

Treatment for pulmonary hypertension can vary significantly, depending on the type of PH. Secondary PH, which is more common, requires treatment of the underlying heart or lung condition. PAH and CTEPH are less common and need specific treatments, including pulmonary thromboendarterectomy (PTE) and/or balloon pulmonary angioplasty (BPA) for CTEPH and a combination of medical therapies for PAH. Lifestyle changes can also help treat some types of pulmonary hypertension and improve quality of life and outcomes when paired with other treatments.

Medications for Pulmonary Hypertension

• Numerous FDA-approved pulmonary vasodilator therapies target specific ways to improve pulmonary circulation and improve right-heart function and symptoms.
• Diuretics ("water pills") help reduce swelling by removing extra fluid from the body.
• For those with low oxygen levels that respond to extra oxygen, supplemental home oxygen therapy can help you breathe better.
• Some PH patients require lifelong blood thinners (anticoagulants) to prevent blood clots.

If pulmonary hypertension does not respond to treatment, a lung transplant may be recommended.

What is Pulmonary Arterial Hypertension (PAH)?

Pulmonary arterial hypertension (PAH) — WHO group 1 pulmonary hypertension — is a progressive, life-threatening disorder characterized by high pressure in the arteries that bring blood to the lungs from the right side of the heart. The arteries become narrowed or blocked, raising right heart pressure and weakening the heart.

Without effective treatment, this condition can progress to right-sided heart failure and cause premature death.

The earlier a definitive diagnosis of PAH is made, the more therapies are available. Early diagnosis and treatment help avoid the need for escalating therapies, such as complex intravenous medications or lung transplantation.

PAH therapy aims to improve symptoms and quality of life and prevent right-sided heart failure and premature death. Current medications for PAH vary by therapeutic target and how each drug is delivered (pills, inhaled, periodic injections, continuous infusion).

Living with Pulmonary Hypertension

Although there is not yet a cure for pulmonary hypertension, treatment and healthy habits can help you feel better and stay active.

Follow Your Treatment Plan

• Take your medications exactly as prescribed.
• Keep all doctor appointments and tests.
• Report new or worsening symptoms to your care team.

Make Healthy Lifestyle Choices

• Stay active: Light exercise like walking can help, but check with your doctor first.
• Eat smart: Choose fresh fruits, vegetables, whole grains and lean proteins.
• Watch your salt and fluids: If advised by your doctor, this helps reduce swelling.
• Don't smoke: Avoid secondhand smoke, too.

Protect Your Lungs

• Get your yearly flu shot and scheduled pneumonia vaccine.
• Avoid high altitudes or air travel without checking with your doctor.
• Let your health care providers know you have pulmonary hypertension.

Care for Your Emotional Well-Being

Living with pulmonary hypertension can be challenging, not just physically, but emotionally too. You might feel overwhelmed, anxious or frustrated at times. That's normal.

You're not alone. Support is available through:

• Pulmonary hypertension support groups (online or in person)
• Talking with a counselor or therapist
• Leaning on family and friends

Stay Connected with Your Care Team

Your care team is here to support you every step of the way. We encourage you to ask questions and share any concerns.

With the right care plan, pulmonary hypertension can be managed so you can live an active and fulfilling life.