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The Hypertrophic Cardiomyopathy (HCM) Center at Tufts Medical Center is the first such treatment and research facility in the Northeast. The HCM Center provides patients and their families with comprehensive, multidisciplinary treatment for this genetic heart disease and specializes in complex cases.
We offer state-of-the-art diagnostic testing as well as the full breadth of treatment options applicable to HCM patients. The general goals of treatment for HCM include minimizing symptoms in everyday life and assessing the risk of and preventing sudden death, as well as recommendations on limits of physical activity and family screening.
All patients referred to the HCM Center are seen by at least one Tufts Medical Center cardiologist, who has extensive expertise in HCM disease management. Depending on the patient, HCM physicians collaborate with preventative cardiologists, interventional cardiologists, electrophysiologists, cardiothoracic surgeons and heart transplant surgeons.
View Martin Maron, MD, Director of the Hypertrophic Cardiomyopathy Center, discuss the warning signs and how HCM is best treated on our tuftsmedicalcenter.tv website.
The foundation of the HCM Center is based on three integral concepts: communication, education, and treatment.
Hypertrophic Cardiomyopathy
Sudden Cardiac Death
Treatment
Cardiac Transplantation
Communication
Education
Research
Appointments
Hypertrophic Cardiomyopathy Center Experts
Hypertrophic Cardiomyopathy
Hypertrophic cardiomyopathy (HCM) is an abnormal enlargement of heart muscle (cardiac hypertrophy) and is the most common genetic cardiovascular disease. Its prevalence in the general population is estimated to be approximately 1:500. Because HCM is a genetic disease, the clinical manifestations are varied and range from asymptomatic to congestive heart failure symptoms, stroke and even sudden cardiac death.
Symptoms of HCM may occur at any age, but risk of sudden death is higher in the young, while heart failure and stroke affect those of more advanced age.
Patients with HCM often have complaints of symptoms such as:
- shortness of breath
- exertional dyspnea
- lightheadedness
- chest pain
Typically, cardiac hypertrophy in HCM is confined to segments of the left ventricular wall and is generally asymmetric. Hypertrophy usually does not develop until adolescence, although childhood cases of HCM are described.
In HCM, the arrangement and pattern of cardiac cells is abnormal. This pattern of cellular disarray is the substrate for the generation of abnormal, potentially lethal arrhythmias.
Approximately one third of patients with HCM have left ventricular outflow tract obstruction. Obstruction is caused when one leaflet of the mistral valve abnormally drags across the left ventricular outflow tract to make contact with the ventricular septum (during the cardiac phase of blood ejection), thereby causing a temporary obstruction to blood flow out of the heart and increased pressures in the left ventricular chamber. This pressure gradient produced by the obstruction can be measured during a patient's echocardiogram.
The majority of patients with symptoms can be treated with pharmacological therapy. Patients with left ventricular outflow obstruction and symptoms that do not improve with medical management may be candidates for cardiac surgery (myotomy/myectomy) or newer procedures such as alcohol septal ablation that eliminate obstruction and thereby improve symptoms.
Sudden Cardiac Death
Sudden cardiac death due to HCM remains an important as well as tragic complication of the disease. The risk of sudden cardiac death ranges between 1-5% per year.
Reported risk factors for sudden cardiac death include:
- Prior resuscitated cardiac death
- Family history of sudden cardiac death
- Personal history of syncope
- Non-sustained ventricular tachycardia on Holter Monitoring
- Massive hypertrophy
- Flat or hypotensive response to exercise
- Possible inducible ischemia
- End-stage phase of HCM (decreased pump function)
Part of our evaluation includes defining an individual’s risk status for sudden cardiac death. All relevant clinical information and risk factors are taken into account when defining a patient’s risk status.
Treatment
Our goal is to provide patients with the most current, evidence-based therapeutic options presently available.
Our first priority is to make sure that our therapeutic recommendations are agreeable and consistent with the patient’s overall interest and well being. Our physicians spend time with each patient ensuring that he or she has a clear understanding of the treatment options, and helping patients feel comfortable with their personal decisions regarding treatment.
Initial treatment will revolve around finding appropriate drug treatment and dosing regimens to best treat symptoms. We work closely with patients to develop a medical regimen that will provide relief of their symptoms.
For patients with left ventricular outflow obstruction accompanied by severe disabling symptoms despite aggressive medical management, we offer several advanced therapeutic options.
These include:
- Alcohol septal ablation
- Cardiac surgery
- Implantation of a dual chamber pacemaker
Cardiac Transplantation
Patients who have severe symptoms despite medical therapy and who have no evidence of left ventricular outflow obstruction may also be candidates for cardiac transplantation.
At Tufts Medical Center we have a very active and experienced cardiac transplantation program that can provide all necessary services and support to those patients with HCM who are deemed transplant candidates.
Communication
We stress the importance that communication plays in the long-term care of patients with HCM. We often see patients who live long distances from our institution, and questions and concerns about the disease and/or the disease management plan can arise between office visits.
Therefore, we strive to develop relationships with our patients that are maintained by responding promptly to patient concerns and questions as they arise. We also work with referring physicians to keep them updated and current on their patients’ status and treatment management issues. Following each visit, a formal letter is sent to a patient’s referring physician.
Education
One of our primary roles is patient education. We appreciate the fact that HCM is a complex disease, often creating uncertainty regarding management. In response to this, during the patient visit we make every effort to answer all questions and concerns that patients may have. We explain all relevant information in a clear, concise and understandable way that will empower patients to live more comfortably and confidently with HCM.
Research
In addition to care of the HCM patient, the Tufts HCM center is also a major research center for patients with this heart disease. Several individuals on the team have contributed to the current understanding of HCM through their work on exertional ischemia, the use of implantable cardioverter defibrillators (ICD) to prevent sudden death, cardiac magnetic resonance imaging for diagnosis and prognosis and the role and management of left ventricular outflow tract obstruction. Our cardiothoracic surgeon has one of the most extensive experiences in performing the surgical myectomy while our interventional cardiologist was the first in the Boston area to perform the alcohol septal ablation for relief of heart failure symptoms in patients with obstructive HCM and continues to be a very active investigator in this area. Finally, we are currently involved in multiple research projects helping to further define risk stratification of sudden death using MRI, determining new genetic profiles in a community based HCM population, as well as exploring novel treatment options to decrease scarring in the heart and improve symptoms. In order to find out more about specific on-going HCM research projects please call the Tufts HCM Center at 617 636-8066.
Appointments
The HCM clinic meets several days a week. Initial visits include an echocardiogram, cardiac MRI and electrocardiogram performed in the morning prior to the clinic visit (or some testing may be completed the day before the visit). All patients will be seen on the initial visit by at least one of the team cardiologists and a comprehensive history and physical, review of medical records, discussion of therapeutic treatment options and screening for risk of sudden death will be performed. As deemed necessary, subsequent evaluations will be made with substantial communication with the patients referring physicians. In addition, arrangements can be made to meet for genetic counseling and other specific social and psychological services.
Appointments as well as any questions concerning a clinic visit can be made by calling Murdena Mills at 617-636-8066 or email: mmills@tuftsmedicalcenter.org
Hypertrophic Cardiomyopathy Center Experts:
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