SALT LAKE CITY , UTAH, USA , June 20, 2017 /EINPresswire.com/ -- Heart patients in Utah and the Intermountain West who suffer from a cardiovascular genetic disorder known as hypertrophic cardiomyopathy (HCM) now have immediate access in Utah to one of only 25 national centers of excellence that specialize in care for this disease.

Intermountain Medical Center Heart Institute’s new Hypertrophic Cardiomyopathy Center has been designated a National Center of Excellence by the Hypertrophic Cardiomyopathy Association (HCMA), joining just two dozen elite programs in the nation to earn this prestigious certification.

The center, which treats adults at Intermountain Medical Center and children at Primary Children’s Hospital, is the only HCMA-recognized center of excellence in Utah and the Intermountain West.

“This is a great honor for our program, and wonderful validation of the work that we’re doing to provide the very best care to families and patients affected by HCM,” said Kia Afshar, MD, director of the Hypertrophic Cardiomyopathy Center at the Intermountain Medical Center Heart Institute.

“We have assembled a group of specialists with expertise and success in treating patients with HCM from throughout the country. This team effort allows us to provide the full range of services that HCM patients in Utah and throughout the Intermountain West need,” Dr. Afshar noted.

The center’s care team members include experts in both adult and pediatric electrophysiology, interventional cardiology, cardiac surgery, advanced cardiac imaging, pediatric heart failure, and transplant cardiology. The center also offers adult and pediatric genetic counseling, social work services, nutrition counseling, pharmacy services, and financial support.

The HCMA works to improve the lives of those with hypertrophic cardiomyopathy, a condition that affects one in every 500 Americans, by preventing untimely deaths and advancing global understanding. The HCMA supports education and advocacy throughout the United States, and works to advance research, understanding and enhance care to patients affected by HCM.

Hypertrophic cardiomyopathy is a heart disease that can cause a number of complications, including sudden death. Hypertrophy means “thickening,” and that’s what happens in cases of hypertrophic cardiomyopathy. Enlargement of cells in the heart leads to thickening of the walls of the ventricles, the heart’s lower chambers. HCM results from a genetic mutation and can lead to symptoms similar to heart failure such as shortness of breath, inability to tolerate exercise, chest pain, dizziness, and fainting.

Frequently, this thickening occurs in the septum, the wall between those chambers, and can reduce or block the flow of blood from the left ventricle to the aorta, creating what’s called hypertrophic obstructive cardiomyopathy. Two-thirds of hypertrophic cardiomyopathy patients develop such an obstruction, according to Dr. Afshar, who’s a heart failure and transplant cardiologist.

“The ventricle is thick and causes the heart to have an abnormal shape and relationship with the mitral valve that can then cause the mitral valve to obstruct blood flow leaving the heart. These people become very short of breath and sometimes have chest pain or dizziness with exertion,” he said.

Hypertrophic cardiomyopathy may also potentially trigger ventricular tachycardia, an erratic heart rhythm that can cause sudden death. Because a high percentage of cases are genetic and symptoms are so varied, if there are symptoms at all, a diagnosis means an individual’s first-degree relatives —parents, children, and siblings — should be screened for the disease, Dr. Afshar said.

Treatments for hypertrophic cardiomyopathy vary, depending on factors including the symptoms and severity of the disease. Some patients can be managed with medications alone but others may need more invasive procedures, such as surgery to reduce the thickness of the septum or septal ablation procedure that causes the thickened wall to shrink.

In 10 to 15 percent of cases, patients may receive a defibrillator to protect against sudden cardiac death. The device is sometimes implanted as a preventive tool in patients with a family history of sudden death and other risk factors for malignant arrhythmias. In end-stage cases, patients whose hearts have become weak due to hypertrophic cardiomyopathy may require a heart transplant.

A center like Intermountain Medical Center Heart Institute’s offers advantages in diagnosis, treatment, and genetic testing, said Dr. Afshar.

Hypertrophic cardiomyopathy is rare enough that a family physician or general cardiologist may not see many patients with the disease. The center’s specialized physicians, however, see it often, talk to other experts about it, stay updated on treatments and research, and are always looking for new and better ways to treat patients suffering from HCM. That specialization improves the quality and consistency of care, said Dr. Afshar.

The new Intermountain Medical Center HCM program offers unique research opportunities, including collection of DNA samples and actively follow consenting HCM patients and family members seen at the center. These samples are housed in Intermountain Healthcare’s active registry, called INSPIRE, which already has over 32,000 biological samples from consenting patients diagnosed with any healthcare-related conditions.

HCM patients and families will also be tracked in our extensive genealogy database of family records, called pedigrees, for over 23 million individuals. Family pedigrees are matched with medical record information from the Intermountain Healthcare system.

Jess C. Gomez
Intermountain Medical Center
801-507-7455
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