Pulmonary Arterial Hypertension

Leading-Edge Therapy for Pulmonary Arterial Hypertension (PAH)

What is pulmonary hypertension?

Pulmonary hypertension (PH) is high blood pressure in the arteries of the lungs that can lead to heart failure. There are five broad types of pulmonary hypertension; they differ in the cause of PH and associated medical conditions. The symptoms of pulmonary hypertension – shortness of breath during exertion, chest pain, fatigue, lightheadedness – are not specific to PH and therefore can go unrecognized or misdiagnosed for years. If you have been diagnosed with pulmonary hypertension, knowing the specific type of PH is critical as there are dramatic differences in treatment and ultimately prognosis. The interdisciplinary team at UC San Diego provides a one-stop destination for accurately diagnosing the type of PH and developing a treatment plan to sustain and improve quality of life.

Pulmonary Arterial Hypertension

UC San Diego Health System is a leader in the diagnosis and treatment of pulmonary arterial hypertension (PAH). PAH is a progressive disorder characterized by high pressure in the arteries that bring deoxygenated blood to the lungs from the right side of the heart. Without effective treatment, PAH can progress to right heart failure and cause premature death.

The symptoms of PAH include shortness of breath during exertion, chest pain, fatigue, lightheadedness, fainting, extremity swelling and accumulation of fluid in the abdominal cavity. Because these symptoms occur in other diseases, misdiagnosis or delayed diagnosis is common.

Diagnosing Pulmonary Arterial Hypertension

UC San Diego has state-of-the-art facilities and experts to accurately diagnose PAH. Diagnosis may include the following tests:

Pulmonary hypertension is typically first detected with an echocardiogram – a common noninvasive test used frequently for screening heart conditions. Physicians then refer their patients to UC San Diego for a pulmonary hypertension workup to determine the exact type of PH. Improper diagnosis and delayed diagnosis are major problems. The earlier a definitive diagnosis of PAH is made, the more therapies are available. With early diagnosis and treatment intervention, we may avoid the need for escalating therapy such as complex intravenous medications or lung transplantation.

Treatment for Pulmonary Arterial Hypertension

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

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

UC San Diego pulmonary vascular specialists make use of all PAH therapeutics to match the best treatment for each patient. Follow up for patients with pulmonary arterial hypertension are individualized depending on treatment response or type of PH.

PAH Clinical Trials and Registry Studies

We offer a variety of clinical trial options for appropriate patients. Some clinical trials are examining novel targets and other trials are investigating improvements to existing targeted therapies. If you are interested, talk to your physician for more information on these trials.

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