The Stanford Adult Pulmonary Hypertension Program recently became one of the first centers in the nation to win top tier accreditation from the national Pulmonary Hypertension Association (PHA). The Stanford Program is now designated a Pulmonary Hypertension Care Center (PHCC).  

The Program is directed by Roham Zamanian, MD, Associate Professor of Medicine (pulmonary and critical care medicine), whose enthusiasm for this new honor is infectious. So too is his concern about the patients with this debilitating and fatal disease whose diagnosis can delayed by months to years because they are not directed to a center with the expertise to properly assess them.

Pulmonary hypertension afflicts women four times more often than men, presenting when patients are in their 40s and 50s and complain of fatigue and shortness of breath. Such symptoms are easily ascribed to other diseases unless the patient undergoes cardiac catheterization, the gold standard diagnostic test for PH. Once diagnosed, patients have a better prognosis than they would have had in the past, but it remains a sobering one.

“Twenty years ago, there were no therapies,” says Zamanian. “The diagnosis was a death sentence with 5-year survival of 30%. Thanks to new therapies developed over the last 15-20 years, today’s 5-year survival is on the order of 60%. But certainly there is no cure.”

New therapies include pills, inhalers, and life-saving constant infusions of intravenous medications directly into the heart for patients who fail first-line therapies. The last-ditch option is lung transplant, which comes with its own set of disadvantages, including a shortage of organs, a need to match blood groups, and shortened survival post-transplant.

Given the dire prognosis for this rare disease, what does this new accreditation by the PHA mean? “It’s huge,” says Zamanian. “By three years ago pulmonary hypertension had become, for lack of a more appropriate term, a ‘sexy’ field to practice in. It has become lucrative because pharmaceutical companies have spent a lot of money and they make a lot of money because the therapies cost between $50,000 and $400,000 annually. So the pharma industry has done a lot of work on marketing to get physicians to prescribe their therapies. The PHA saw a need to measure quality assurance in the programs popping up all over the place as well as a way to accredit physicians. So they developed criteria and benchmarks for centers of excellence.”

Zamanian submitted an application to demonstrate that Stanford’s Program had the clinical expertise, capacity, in-house transplant, backup from multiple services, including cardiology and anesthesiology, and having the institutional expertise, especially in pulmonary and critical care. Stanford was chosen as one of the pilot program following the rigorous application process and was site-visited in March. The result? “The PHA felt Stanford’s is what programs should aspire to be,” Zamanian says with pride in his voice. “It means a lot to us to be one of the first centers in the world to be given this designation of Comprehensive Care Center. It really validates what we have done.”

In the seven years since Zamanian became director of the Program, it has grown “from 2 physicians and 1 nurse practitioner to 5 physicians, 1 fellow trained in pulmonary hypertension, and an excellent group of nurses and staff, including research coordinators: 20 people in all. This is quite large for a rare disease.” Two of the physicians are basic scientists 80% of the time who exemplify translational medicine by bringing their lab-based discoveries to the bedside the other 20% of their time.

How does the newly-accredited Program fit into the tripartite mission of academic medical centers: patient care, teaching, and research?

Patient Care

For the approximately 100,000 patients with pulmonary hypertension in the United States, PHA accreditation means that “they can choose programs that have undergone quality measurements to be sure they have capacity and knowledge. The PHA will be collecting outcome data and eventually grading programs’ outcomes as well.”

As an example of the difference between highly qualified centers and others, Zamanian mentioned that patients are sometimes diagnosed and treated on the basis of an echocardiogram, which can misdiagnose some patients. “All patients need cardiac catheterization for a correct diagnosis. Patients who present to a PHA-qualified center will know that they are more likely to be correctly diagnosed and given the latest treatments. Being treated at an accredited center may also ease the care of PH patients, perhaps reducing their healthcare costs by eliminating inappropriate care.”

Teaching

On the educational level, what might this accreditation mean to potential fellows? First, according to Zamanian, Stanford has the only curriculum-based fellowship in the US and has graduated a total of 15 fellows; all but one who graduated in the past 7 years have gone into academic careers and have become major leaders in their field. By joining academic centers in San Diego, New Orleans, Seattle, British Columbia, New Mexico, and other locations, Zamanian says that he “feels we’ve seeded some programs of excellence that patients need in several parts of the country.” The one graduate who did not go into academics joined Actelion Pharmaceuticals and is working there to bring new therapies for PH to market.

On another educational front, Zamanian is pursuing the approval of a new pulmonary vascular subspecialty from the American Board of Internal Medicine and the Accreditation Council for Graduate Medical Education.

Research

The third part of the tripartite mission is research. What kind of research is Stanford doing into PH, its causes, treatment, prevention, and cure?

“We do cutting-edge clinical research. I started a biobank in 2007 and we’ve collected over 20,000 samples (of blood, urine, exhaled breath) from patients so that we can study them in a transformative way. We have a database of over 1,000 patients, each having over 300 different tests captured. We have balanced our clinical trials approach. Most centers just do trials with pharmaceuticals, but we have made room to bring our own therapeutics that we have developed in the lab to the clinic.”

To Sum Up

In Zamanian’s words: “This accreditation is important to us, not only because of the regional and the national recognition, but also the realization that we are a program that personifies the things that the PHA wants to see in its top centers.”