Pulmonary Hypertension Program Receives Comprehensive Care Center Status

Accurately diagnosing pulmonary hypertension has eluded physicians for years. With symptoms such as shortness of breath and fatigue, it is often misdiagnosed as asthma, and inadequately treated. By the time patients are referred to the Vera Moulton Wall Center for Pulmonary Vascular Disease, their life expectancy is in the single digits.

The Stanford Adult Pulmonary Hypertension Program, which recently received accreditation as a comprehensive care center by the Pulmonary Hypertension Association, includes a team of physicians, advanced practice providers, patient and nurse coordinators and a medical social worker who provide definitive diagnosis and expert management of these complex patients.

Accreditation is based on a program's overall commitment to patients, breadth of health care professionals involved, scope of services provided and rigorous research involvement in the disease.

"The PHA recognized that we are not only caring for our patients well, but we also provide access to transplant, lifesaving therapies and experimental treatments," said Roham Zamanian, MD, medical director for the Adult Pulmonary Hypertension program. "We are also conducting research and educating a future generation of cardio-pulmonary specialists."

"The comprehensive center designation was born out of a concern for quality and excellence in research, and as a way to delineate those centers that are doing best by the patient," said Juliana Liu, RN, NP, an adult nurse practitioner for the program. "We're very excited to be one of the first centers in the country to get this accreditation. It is really a recognition of the work we've been doing for many years."

What is PH?

In pulmonary hypertension, due to various reasons, the blood pressure in the pulmonary (lung) arteries is abnormally high. In pulmonary arterial hypertension, the inner lining of the arteries continue to grow, leading to increased resistance and pressure. Regardless of the type of pulmonary hypertension one has, the right side of the heart enlarges due to the increased workload of pumping blood against this resistance. Left untreated, the disease can lead to death from heart failure.

Pulmonary hypertension disproportionately affects women of childbearing age, and occurs in about one or two individuals in a million. The risk is substantially higher for people with certain autoimmune diseases such as scleroderma or lupus, HIV, congenital heart disease or liver disease; and for those who have used methamphetamines or cocaine.

"Twenty years ago, there was a 37 percent survival rate at five years for patients with pulmonary hypertension," said Zamanian. "Today, at this center, we have a 70 to 75 percent survival rate at five years."

Those promising results are achieved despite treating "the sickest of the sick," said Zamanian, an assistant professor in the Division of Pulmonary and Critical Care Medicine. "Half of our new patients are on track to die within a year. By the time individuals with pulmonary hypertension become symptomatic, 60 to 70 percent of their lung vasculature has already been injured."

Burgeoning treatments

While there is still no cure for pulmonary hypertension, an arsenal of medications has extended the lives of many. Prior to 2000, there were only two treatments available, both of which must be administered intravenously, 24 hours a day, seven days a week. Because these IV medications require a high degree of expertise to manage, many physicians do not prescribe them, or only use them in very low, homeopathic doses, preferring instead to use one of 15 new oral medications that became available starting in 2001.

These oral vasodilators work to expand blood vessels in the lungs. But each works a little differently, and some can be contra-indicated in certain patients. Liu finds she often achieves better results using the intravenous medication Flolan for many of her patients, despite the difficulty in administering it.

"We now have an armamentarium of therapies to give, but using them appropriately requires a lot of expertise," said Zamanian. This expanded pharmacopeia also allows prescribing physician to switch or add new medications as drugs lose their potency over time. Determining which drug to initiate in any one patient requires teasing out the etiology of a patient's disease through a litany of tests, said Liu. "Accurate dosing also requires an understanding of the characteristics of the drugs," she said. "There are physicians who are starting patients inappropriately or not titrating them appropriately on their mediation."

High-volume clinic

Part of the Stanford Adult Pulmonary Hypertension's expertise in medical management comes from pure patient volume; the Stanford Center sees several hundred patients every year, for a disease that affects relatively few.

"We probably see the highest volume of patients with this disorder than any other center," said Zamanian. As such, the program has become a teaching ground for physicians interested in pulmonary hypertension. To date, the program has trained 15 fellows in the complex care of patients with pulmonary hypertension.

This large patient volume also fuels research into novel therapies. As the scientific understanding of pulmonary hypertension's molecular underpinnings grows, the arsenal of available drugs deviate from its current vasodilator focus. The Stanford bench is active in developing a generic, lower-cost pill for the treatment of pulmonary hypertension, as well as discovering the use of a novel anti-inflammatory agents that are curative in animal-based PH models.

"We're developing therapies that we think are going to be disease modifying," said Zamanian. "These are not vasodilators, but rather anti-proliferative, anti-inflammatory agents. Ultimately, we want to strive to come closer to a cure. Until then, we hope to deliver the best care possible for these patients."