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 by cause and associated medical conditions. The symptoms of pulmonary hypertension – shortness of breath during exertion, chest pain, fatigue and 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 the treatment and 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 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-sided 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 Health System for a pulmonary hypertension workup to determine the exact type of PH. Improper diagnosis and delayed diagnosis can be detrimental. 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 current 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 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).
UC San Diego pulmonary vascular specialists make use of all PAH therapeutics in order to determine the best treatment for each patient. Follow up for patients with pulmonary arterial hypertension is individualized, depending on treatment response and type of PH.
PAH Clinical Trials and Registry Studies
We offer a variety of clinical trials for appropriate patients. Some clinical trials examine novel targets, while others investigate 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 better understanding rare diseases such as these. Through registry studies, physician-scientists are able to pool data from multiple medical centers to better understand disease progression and outcomes for different therapies.