When you have high blood pressure in your lungs, it’s called pulmonary arterial hypertension (PAH). Because symptoms can include fatigue and shortness of breath, this chronic, life-altering disease can make even simple tasks like climbing stairs difficult. While doctors and researchers continue to work on a cure, several treatment options are available that greatly improve the quality of our patients’ lives and delay the progression of the disease which can become life threatening. With the right care and coping strategies, many of our patients are able to lead full lives after diagnosis.
The Leading Pulmonary Hypertension Treatment Center in the Region
While the incidence of PAH is rare (for every million people in the United States, there are only one to two cases), every year hundreds of PAH patients and their families come to the John Ochsner Heart and Vascular Institute (JOHVI). Here, at the largest pulmonary hypertension treatment center in the Gulf South, they find the answers they seek as well as the relief they need to live with this challenging disease.
Meet Your Team
The Ochsner PAH team includes intensely dedicated cardiologists, pulmonologists, advanced practice providers, and outpatient nurses passionate about caring for patients with pulmonary hypertension. In addition, a large number of highly skilled inpatient nurses in the Transplant Step-Down Unit and Cardiac-Medical ICU have a wealth of experience treating this disease. As part of Ochsner Health System, we have also developed strong relationships with world-renowned experts in other subspecialties, including cardiac anesthesia, palliative care, rheumatology, nephrology and pediatric cardiology, to name but a few. Our social worker team works closely with home health care agencies and Ochsner Total Health Solutions to get our patients the care and medical equipment they need to improve their lives. We also partner with several specialty pharmacies to provide 24-hour support for patients who need it.
Dizziness upon standing, climbing stairs, straightening up from a bent position, or even while just sitting
Fainting or near fainting
Loss of energy/fatigue
Swollen ankles, legs and abdomen
Palpitations or racing heart beats
What Causes PAH?
PAH is a disease that does not discriminate – our patients include people of all ages and backgrounds. Often the cause of the pulmonary hypertension is unknown. It may be inherited, or associated with another disease process. Some of these associated conditions include:
Left-sided heart disease
Chronic lung disease
Congenital heart disease
Blood clots in the lungs
Sickle cell disease
Sleep disorders, such as sleep apnea
Connective tissue diseases, such as scleroderma or lupus
The Right Diagnosis
Because PAH shares symptoms with anxiety, asthma, COPD and other conditions, patients are often misdiagnosed. The Ochsner Pulmonary Hypertension Program offers a comprehensive evaluation process to determine if you have PAH, ensuring the most effective course of treatment moving forward.
Diagnostic Tools Include:
Pulmonary function test (measure lung function)
Ventilation perfusion lung scan (measures air and blood flow to the lungs)
Individualized Treatment Plans
Once a diagnosis of PAH has been confirmed, our team works with each patient to develop an individualized treatment plan. We offer both clinic and hospital-based care, and use all medications currently approved by the FDA for the treatment of pulmonary hypertension.
Treatments We Offer Include:
Your doctor may give you prescriptions to help improve the blood flow through the lungs, reduce fluid levels that put pressure on the heart, as well as blood thinners to prevent blood clots. One important class of oral medications are the pulmonary vasodilators; pulmonary hypertension-specific medications which ease symptoms and increase exercise tolerance.
Intravenous (IV) Medicines/Subcutaneous Treatments
IV medications and subcutaneous treatments (delivered through a portable infusion pump) can be extremely effective at opening up your blood vessels. The treatment helps to ease symptoms of PAH, including chest pain and shortness of breath and can increase exercise tolerance and improve survival.
Oxygen relieves shortness of breath and helps relax the blood vessels in the lungs.
Inhaled Treprostinil and Inhaled Iloprost both ease symptoms and improve exercise capacity.
Pulmonary Thromboendarterectomy (PTE)
PTE is a surgical procedure that, in select patients, removes blood clots in the lungs and may potentially cure the patient’s pulmonary hypertension. Through this procedure blood clots can be removed from the arteries in your lungs to help you breathe easier.
As the only lung transplant program in Louisiana and Mississippi, the Ochsner Lung Transplant Team has performed more than 300 of these life-saving procedures. Patients with PAH are referred early to be evaluated for lung transplantation, most often at the time of initiation of parenteral therapy. The PAH and Lung Transplant teams work closely together to care for our shared patients both in the outpatient and inpatient arenas. Learn more about our program.
Pulmonary rehabilitation is a multidisciplinary program for patients with PAH and other chronic respiratory impairments. Programs are individually tailored by our staff to meet your unique needs. Learn more.
PAH Support Group
If you or a family member has PAH, chances are you can benefit from the experiences of others who have learned to manage the physical and emotional aspects of this disease. Ochsner is home to an active patient support group which provides both education and invaluable support to patients and their families. View Support Group dates and information.
Research and Clinical Trials
Ochsner has a long history of enrolling PAH patients in registries and clinical trials not available elsewhere in the region. Ochsner has a strong Cardiovascular Research Division, which has been committed to providing our PAH patients with access to clinical trials and registries. What’s next is right here at Ochsner. Talk to your doctor to see if one of these trials is right for you. View clinical trials.
The Pulmonary Hypertension Program at the John Ochsner Heart and Vascular Institute (JOHVI) is the primary referral center for Gulf Coast patients with PAH. While many patients are referred to us by general practitioners, cardiologists, and pulmonary specialists, self-referrals are also welcome. Call 504-842-4721 to refer a patient.
It is never too early to refer a patient with suspected or diagnosed PAH. Proper treatment has been found to extend life and improve quality of life. However, early intervention remains the key to maximizing treatment efficacy and patient outcomes. When an accurate PH diagnosis accompanied by appropriate treatment is achieved early in the disease progression (functional classes I and II), patients report fewer limitations and greater quality of life.
Please also consider referring:
Patients on oral therapy without significant improvement in symptoms or functional capacity, or continued decline despite addition of oral pulmonary vasodilator therapy.
Functional class III or IV patients.
Patients with Connective Tissue Disease with PASP>35 on echo