Image courtesy of Blausen.com staff. “Blausen gallery 2014”.

For nearly half a century, BWH has been at the forefront of breakthroughs in understanding and treating hypertrophic cardiomyopathy (HCM), the most common inherited cardiovascular disorder.

In the 1950s, Eugene Braunwald, MD, founding chairman of the TIMI Study Group, defined the pathophysiology of the disease, which is characterized by an excessive thickening of the heart muscle with no obvious cause. In 1990, Christine Seidman, MD, director of the Cardiovascular Genetics Center, and Jon Seidman, PhD, discovered the genetics of HCM, a disease that runs in families. Today, Carolyn Ho, MD, medical director of the Cardiovascular Genetics Center, is bringing genetics into clinical practice through a series of cutting-edge clinical trials for HCM patients.

It’s no surprise that BWH is now leading the way in using a more systematic approach to identify and treat patients with HCM, a condition that affects about one in 500 people.

“So many of the decisions we have to make in the care of HCM patients are without great literature to guide us,” said Neal Lakdawala, MD, of BWH’s Cardiovascular Genetics Program.

Although HCM is an important cause of sudden cardiac death, heart failure and stroke, most patients with the condition experience normal longevity. Great progress has been made through research in helping to prevent sudden death—the most severe possible outcome of HCM—with implantable defibrillators and advanced cardiac imaging.

To begin understanding the best way to manage stable HCM patients, BWH’s Cardiovascular Genetics team developed a Standardized Clinical Assessment and Management Plan (SCAMP) focused on the most effective use of cardiac imaging.

A SCAMP is an approach to quality improvement that aims to optimize outcomes and resource utilization through standardization of care and understanding reasons for variation. While SCAMPs appear similar to other care pathways, they allow physicians to vary from the care plan so long as they state a reason.  The Cardiovascular Genetics team worked with BWH’s SCAMP team to create a practice algorithm and begin capturing data to develop a protocol to improve the efficiency of repeat imaging.

“We wanted to use a SCAMP to make sure that we are doing our very best job providing quality care for our patients,” said Lakdawala.

Data collection and a pilot for the SCAMP began in April 2013, and the process was fully implemented in July 2013. To date, the group has achieved great success with the results. A key finding was that repeating an echocardiogram annually for stable patients isn’t changing the way these patients are managed.  Current guidelines advise annual echos for HCM patients, but there isn’t substantial evidence-based data to support that, as optimal management of stable patients is poorly defined.

“The results of the SCAMP are allowing us to decrease repeat imaging and testing where it isn’t necessary,” said Lakdawala. Rather than repeat the echo annually for stable patients, the team is now looking at performing the echo every two years.

Another key finding is especially important for identifying patients. In spite of HCM being recognized as a disease that affects families, many at-risk family members aren’t being appropriately followed. “We learned that many relatives of patients aren’t getting testing, even those at high risk,” Lakdawala said. “The SCAMP gave us numbers to support our anecdotal experience.”

Genetic testing can influence the likelihood that family members would come in for evaluation, an important step to getting the care they need for this potentially fatal condition.

About 350 to 400 patients are under the care of BWH physicians, either because they have HCM or a family history of the condition and therefore are at high risk.

Lakdawala said the SCAMP experience has been a positive one for the team. “Everyone embraced this process enthusiastically,” he said. “I’m optimistic that we will continue our work and that our results will ultimately influence not only BWH patients, but also have implications for many other patients living with HCM.”

The team presented preliminary results of the SCAMP last year at an American Heart Association conference, and received positive feedback from attendees.

Learn more about SCAMPs at BWH

BWH is leading the way nationally in the use of SCAMPs at adult hospitals.

A good SCAMP focuses on a condition in which uncertainty in management exists or an area in which guidelines do not exist or are not well followed, and practice variation exists. A condition with room to improve outcomes, or one with multiple decision points involving diagnostic tests, treatments and other management conditions, also make for good SCAMPs.

Those interested in learning more about how to implement a SCAMP should contact: SCAMP Team Medical Director Jeffrey Greenberg, MD, at jogreenberg@partners.org, or SCAMP Team Program Manager Roya Ghazinouri, PT, DPT, MS, at rghazinouri@partners.org.