CTEPH & Pulmonary Hypertension Care

UC San Diego Health is a global leader in the diagnosis, treatment and research of pulmonary hypertension, including pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH).

Our pulmonary vascular disease program, led by Nick Kim, MD, includes pulmonary and cardiovascular medicine specialists, cardiothoracic surgeons and anesthesiologists. They are all dedicated to providing safe, effective care tailored to your needs.

What is Pulmonary Hypertension?

Pulmonary hypertension is high pressure in the blood vessels that carry blood into the lungs. This can strain the lungs and heart, and lead to serious problems.

What Causes Pulmonary Hypertension?

The cause is sometimes not known. But it's most often caused by another health problem. In many cases, controlling this problem can help prevent or control pulmonary hypertension. Some of the most common causes of pulmonary hypertension in adults are:

• Lung conditions, such as chronic obstructive pulmonary disease (COPD), advanced bronchitis, cystic fibrosis, or pulmonary fibrosis
• Liver disease
• Blood clots in the lungs
• Left-sided heart failure
• Sleep apnea
• Other conditions, such as scleroderma, lupus, or sickle cell disease
• Some medicines
• Genetic disease

Symptoms of Pulmonary Hypertension

Symptoms may come on suddenly. Or, they may come on slowly over time. Symptoms can include:

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

Diagnosing Pulmonary Hypertension

Diagnosing pulmonary hypertension can be difficult because many of its signs and symptoms are similar to other lung diseases, like asthma and chronic obstructive pulmonary disease (COPD). UC San Diego Health experts can evaluate your condition and provide an accurate diagnosis.

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

Your diagnosis may include the following tests:

• Echocardiogram
• Cardiac catheterization
• Pulmonary angiogram
• CT Scan
• MRI
• Pulmonary function tests
• Cardiopulmonary exercise stress test

What Is Chronic Thromboembolic Pulmonary Hypertension (CTEPH)?

CTEPH, a rare type of pulmonary hypertension (high blood pressure in the lungs), arises from repeated or unresolved pulmonary embolisms or clots in the pulmonary arteries.

When these blood clots in the lungs don’t dissolve, they can cause scar-like tissue to form. That can clog and narrow the arteries, leading the blood pressure to go up.

PTE — Advanced Treatment for CTEPH

Designated a Center of Excellence by the American College of Chest Physicians, our program has the highest success rate worldwide for pulmonary thromboendarterectomy (PTE) surgery, a complex procedure used to treat CTEPH.

Until we developed PTE surgery at UC San Diego Health, the treatment for CTEPH was lung transplantation. PTE is now saving thousands of lives worldwide.

Our team also offers balloon pulmonary angioplasty (BPA) and medical therapy for individuals with CTEPH who are not candidates for PTE surgery.

What Is Pulmonary Arterial Hypertension (PAH)?

PAH — another kind of pulmonary hypertension — is a progressive, life-threatening disorder characterized by high pressure in the arteries that bring deoxygenated blood to the lungs from the right side of the heart. The arteries become narrowed or blocked, raising blood pressure and weakening the heart.

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

UC San Diego Health is a leader in diagnosing and treating pulmonary arterial hypertension.

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.

Advanced Treatment for PAH

UC San Diego Health is a hub for advanced therapies for pulmonary arterial hypertension. We were actively involved in the clinical trials for all current FDA-approved PAH therapies. Today, we remain engaged in clinical trials examining new and improved treatments for PAH and other forms of pulmonary hypertension.

PAH therapy aims to improve symptoms and quality of life and prevent heart failure and premature death. Current medications for PAH vary by therapeutic target and how each drug is delivered (oral, inhaled, subcutaneous or intravenous).

Our pulmonary vascular specialists use all PAH therapeutics to determine the best treatment for you. Our follow-up services are individualized, depending on your response to treatment.

If PAH does not respond to treatment, we work closely with our lung transplantation program for evaluation and placement on the transplant list.

Clinical Trials

UC San Diego Health participates in registry studies, which are important for better understanding rare diseases such as PAH and CTEPH. Through registry studies, physician-scientists can pool data from multiple medical centers to understand disease progression and outcomes for different therapies.

We offer a variety of clinical trials for appropriate patients. If you are interested, talk to your physician for more information about these trials.