Overview

Hypertrophic Cardiomyopathy (HCM) is a heart condition in which the heart muscle is thickened for unexplained reasons. It affects up to 1 in 200 individuals of all ages and is often inherited or genetic.

The UW Medicine Hypertrophic Cardiomyopathy Clinic was founded in 2009 to improve the lives of patients with HCM throughout the Pacific Northwest. Our mission is to provide comprehensive, high-quality care for HCM patients and their families through guideline-directed care, personalized treatment approaches, and innovative research.

The UW Medicine HCM Clinic includes a multidisciplinary team of experts with extensive experience in managing all aspects of HCM care. This includes HCM specialists, electrophysiologists, interventional cardiologists, heart failure / transplant specialists, cardiac surgeons, geneticists, cardiovascular imaging specialists, sports medicine providers, cardio-obstetricians, and pediatric cardiologists.

Patients and families with HCM will face many challenges and uncertainties along their journey. We believe that each HCM heart is unique in its anatomy and physiology, requiring individualized treatment approaches. To provide the best care for our patients, each person is evaluated and treated using an inclusive, patient-centered, and integrated approach as we establish an ongoing partnership in care.

UW Medicine seeks to actively advance HCM care through clinical, translational, and basic science research. This includes participation in multi-center clinical trials of novel medical therapies, including the pioneering use of mavacamten and other cardiac myosin inhibitors.

At UW Medicine, we focus on three main aspects of HCM care: (1) management and treatment of symptoms; (2) assessment and prevention of abnormal heart rhythms and sudden death; and (3) screening of family members using genetic testing or imaging.

Obstructive HCM

Many patients with HCM have symptoms related to the obstruction of blood flow leaving the heart. Fortunately there are several effective treatment options available to improve obstruction-related symptoms, including medications, procedures, and surgeries. We work to educate patients about their treatment options and tailor an approach to each individual based on shared decision making.

For some patients, treatment with cardiac myosin inhibitors may be the right option. UW Medicine participated in the clinical trials that brought these medications to FDA approval, and we have long term experience in dosing and monitoring these agents.

For patients with advanced obstruction-related symptoms, UW Medicine has decades of experience with both alcohol septal ablations and surgical myectomy. UW Medicine is considered among the highest-volume centers in the US for these procedures, and on grouped analysis was among the centers with the best surgical and procedural outcomes.

Risk Assessment and Rhythm Management

Patients with HCM may be at risk of developing abnormal heart rhythms and this risk may change over time. We take a proactive approach to risk assessment with surveillance monitoring plans tailored to each patient’s risk and goals.

For patients who are thought to be at increased risk of abnormal heart rhythms, implantable cardioverter-defibrillators (ICDs) – both traditional and subcutaneous – offer protection against sudden death. The decision to proceed with an ICD is rarely simple, and our team of electrophysiologists (heart rhythm experts) have extensive experience counseling patients on these risks using a shared decision-making approach.

Abnormal heart rhythms such as atrial fibrillation are common in HCM, and our team of electrophysiologists have extensive experience managing medications and / or performing ablations on HCM hearts.

Family Screening

HCM is defined as “unexplained” heart muscle thickening, but in many cases it is inherited and runs in families. We recommend patients consider family screening, using either a genetic testing or imaging-based approach.

A genetic cause of HCM can be found in about 40-50% of all HCM patients, though the yield is higher among certain HCM subtypes. If a genetic cause is identified, these results can be used to test other family members to see if they are also at risk of developing HCM.

UW Medicine has a team of medical geneticists that are focused on inherited forms of heart disease and can help navigate the testing process, the test results interpretation, and any additional family testing that may be needed.

For children needing screening or those who are identified as having a genetic change capable of causing HCM, we partner with pediatric cardiologists at Seattle Children’s Hospital for ongoing management and surveillance.

Advanced HCM

Most patients with HCM have a normal life expectancy. However, a small percentage of HCM patients have a progressive course with development of advanced heart failure or other advanced symptoms.

UW Medicine has a team of Heart Failure / Transplant cardiologists focused on the care of advanced heart failure. UW Medicine is among the highest volume cardiac transplantation centers in the United States.

Cardio-Obstetrics and HCM

HCM can affect patients of all ages. For women with HCM who are of child-bearing age, pregnancy may be desirable but also a source of anxiety and concern.

Fortunately, most women with HCM are able to have successful pregnancies with low maternal and fetal risks. UW Medicine has a multi-disciplinary team of Cardio-Obstetric providers that work with HCM patients on initial pre-conception counseling, provide maternal and fetal monitoring throughout pregnancy, and a develop a delivery plan tailored to each woman’s needs.

Services Provided

The UW Medicine HCM Clinic provides:

• Consultation by a team of clinicians with expertise in diagnosing and managing HCM and other, related heart muscle disorders.
• Ongoing follow up with personalized treatment recommendations.
• Medical geneticists specializing in inherited heart disorders, with full spectrum genetic testing services to confirm a diagnosis and assist with family screening.
• Surgical septal reduction performed by experienced surgeons, and minimally invasive septal reduction using alcohol ablation. UW Medicine is a high-volume referral center for both surgical myectomy and alcohol septal ablation.
• An electrophysiology team with expertise in pacemakers and implantable cardioverter-defibrillators (ICD) to prevent sudden cardiac arrest, and in performing ablations for atrial and ventricular heart rhythm disturbances.
• Echocardiographic examinations tailored for HCM patients, including 3D, strain, and treadmill exercise testing.
• Advanced cardiovascular imaging techniques including cardiac MRI and PET scans to detect scarring / fibrosis or inflammation.
• For patients with advanced heart failure symptoms, evaluation by Heart Failure / Transplant specialists.
• Close collaboration with UW Medicine Cardio-Obstetrics clinic for pre-conception counseling and to ensure optimal maternal and fetal outcome for pregnant women with HCM.
• Close relationship with Seattle Children’s Hospital for HCM screening and evaluation of children and to provide smooth transition of care from pediatric to adult cardiology.
• Ongoing research studies that aim to improve the diagnosis and treatment of HCM.