Improving cardiomyopathy care using multidisciplinary expertise

New therapies and advances in genetic testing have changed the landscape of cardiomyopathy care, and specialized expertise can mean more healthy years for patients. The Inova Reinsch Pierce Cardiomyopathy Program provides cardiovascular care to patients with all forms of cardiomyopathy including:

• Arrythmogenic
• Dilated
• Hypertrophic
• Restrictive

Whether a cardiomyopathy is common – one in 500 Americans has hypertrophic cardiomyopathy, for example – or exceedingly rare, Inova’s program brings together a specialized, multidisciplinary team of experts dedicated to diagnosing and treating this complex condition.

Benefits of a comprehensive approach

The cardiomyopathy team at Inova includes cardiologists, nurse practitioners, patient care coordinators, imaging experts, research coordinators, registry experts and data analysts, all working together to diagnose patients, determine the best treatment plan, execute that plan and evaluate patient outcomes. In addition, the team collaborates closely with other specialists such as electrophysiologists, cardiac surgeons and advanced lung disease specialists based on the individual patient’s needs.

Available treatment options include:

• Medication to improve symptoms
• Ablation procedures
• Device implantation
• A variety of surgical options

Inova also offers a robust clinical research program, enabling the cardiomyopathy team to offer patients leading-edge therapies, new devices and groundbreaking.

“Inova strives to provide multidisciplinary care for a wide variety of complicated cardiomyopathy disease states, which includes inflammatory cardiomyopathies, infiltrative cardiomyopathies and inherited cardiomyopathies,” said Mitchell Psotka, MD, PhD, Section Chief, Heart Failure and Transplant at Inova Schar Heart and Vascular. “Our team members have highly integrated partnerships with other medical and surgical subspecialists in rheumatology, dermatology, nephrology, hepatology, pulmonology and endocrinology. These multidisciplinary care groups are needed to provide the best care for conditions such as hypertrophic cardiomyopathy, genetic cardiomyopathies and inflammatory or infiltrative cardiomyopathies such as sarcoidosis, Chagas disease and cardiac amyloidosis.”

Should a patient’s cardiomyopathy progress beyond the point where medications can control it, Inova offers expertise in advanced heart failure, mechanical circulatory support and heart transplant. Inova’s team performs more than 40 heart transplants every year.

Hypertrophic cardiomyopathy clinic provides customized care

This condition is characterized by hypertrophy, an abnormal thickening of the heart wall muscle that can impair the heart’s ability to relax appropriately and can obstruct blood flow from the heart, resulting in symptoms of heart failure. Patients with hypertrophic cardiomyopathy are also at risk for arrythmias including those associated with sudden cardiac death. It’s crucial to not only manage these patients’ symptoms but also for a cardiomyopathy expert to risk stratify them appropriately, working in conjunction with an electrophysiologist, to protect them from sudden cardiac death.

At Inova’s hypertrophic cardiomyopathy clinic, the team provides:

• Comprehensive, noninvasive testing for accurate diagnosis and risk stratification
• Access to the latest medical, surgical and procedural treatment
• Opportunities for participation in clinical research studies

Cardiovascular genetics – key to cardiomyopathy care

Because cardiomyopathy often has a genetic component, Inova’s cardiomyopathy program includes cardiovascular genetic testing and counseling as part of the initial evaluation process. About 25% of patients with dilated cardiomyopathy will carry a genetic mutation that is the cause. If they have a family history of cardiomyopathy, that percentage can be as high as 50%. In hypertrophic cardiomyopathy, the presence of a genetic mutation that causes the disease is seen in about 40 to 60% of patients.

Using a team approach, patients see both an advanced heart failure cardiologist and a genetic counselor at the initial visit. Working together, the team takes a comprehensive family history that includes a three-generation pedigree. “That really allows us to understand the health problems that have been going on in the family and determine whether there are any patterns that may suggest an inherited disease, cardiomyopathy or cardiovascular condition,” said Palak Shah, MD, MS, Director of the Inova Cardiovascular Genomics Center and Medical Director of Mechanical Circulatory Support for Inova Fairfax Hospital.

Next, the team educates the patient and their family about the benefits and limitations of genetic testing. The program facilitates testing, if the patient or family members are interested. When the results are available, the team meets with the patient and family members to discuss the test results’ implications, both for the patient and family members.

“Through that process, we often end up managing not just an individual, but an entire family that is at risk for a genetic disease or has inherited the same genetic mutation,” Dr. Shah said. “That’s an important component of the multidisciplinary care we provide here.”

Modern cardiovascular genetic testing can look at as many as 150 genes in a single panel, making it more likely that testing can identify a potential genetic cause of the cardiomyopathy. For patients with more complex cardiomyopathies or strong family histories of genetic disease, whole genome sequencing can also be used to pinpoint a genetic cause.

The result of identifying a genetic cause is more than just identifying familial risk. New medications are now becoming available that specifically target genetic mutations of a given cardiomyopathy. And thanks to the genetic information captured through research across the world, it’s sometimes possible to predict how, for example, a dilated cardiomyopathy caused by a certain genetic mutation will behave over time.

“By understanding the specific mutation, we are better able to understand which patients are more likely to have aggressive disease – which patients are more likely to develop arrhythmias, for example – and which patients may benefit, or families may benefit, from earlier intervention,” Dr. Shah noted.

Improving patient outcomes

Having access to specialized expertise and guideline-directed medical therapy and risk stratification can give patients a decade or more of normalized heart function. Inova’s cardiomyopathy team, from outpatient heart failure clinics to hospital-based care and advanced therapies for end-stage disease, provides outstanding outcomes for patients.

“Our philosophy aligns with Inova’s mission as a health system – to provide the best patient care, every time, every touch,” Dr. Psotka said. “By incorporating multidisciplinary subspecialty expertise, we are best able to provide that for our patients with cardiomyopathy.”

Learn more about the Inova Reinsch Pierce Cardiomyopathy Program.