Providence Cardiomyopathy Program
Our multidisciplinary Hypertrophic Cardiomyopathy (HCM) center will provide the most comprehensive and cutting edge care for patients and their families having either a diagnosis of, or concern for HCM. We have assembled at team of physicians and healthcare professionals from some of the world’s best institutions.
Our collective goal is to deliver individually tailored therapies for each of our patients. Since HCM is often a genetically mediated condition, we provide access to genetics testing to our patients and their families, if needed. Furthermore, we have access to the national community of HCM specialists and are able to direct family members who do not live locally to their closest reputable HCM center should they require further management.
We pride ourselves in ensuring that our patients will come away from a consultation empowered by their understanding of HCM. They will be reassured by the knowledge that there is a team of professionals highly trained in the management of their specific condition providing access to the most up to date, evidence based treatments available.
Hypertrophic Cardiomyopathy (HCM) is a complex, predominantly genetically mediated heart disease, affecting approximately 1 in 500 individuals. The disease is characterized by excessive thickening of the heart muscle, most commonly in the septum, which is the muscular wall that separates the lower chambers of the heart. The severity of symptoms and progression of HCM vary greatly between each patient. The most common symptoms include chest pain, shortness of breath, palpitations (forceful or irregular heart beating), lightheadedness, and syncope (fainting).
Testing in HCM usually includes, but not limited to, electrocardiogram (ECG), echocardiography, stress echocardiogram, and cardiac MRI.
There are two major types of HCM:
- Obstructive HCM
- Obstructive HCM is defined as the partial obstruction (or blockage) of blood flow out of the heart. This is a result of abnormal motion of the mitral valve, causing it to come in contact with the thickened septum. This results in increased pressure within the lower chamber of the heart and a possible drop in blood pressure.
- In some patients, this obstruction of flow is present at rest, while in others it occurs only during exercise. For this reason, an exercise stress test must be performed.
- For some patients, the obstruction of flow can be treated effectively with medications. However, in some cases medications are not effective and invasive therapies, such as open heart surgery or catheter based interventions, are needed to adequately to relieve the obstruction.
- Non obstructive HCM
- Non-obstructive HCM is characterized by thickened heart muscle without obstruction to blood flow at rest or with exercise.
- Non obstructive HCM is adequately managed with medications alone and invasive therapies are not indicated.
- A small percentage of patients with HCM are at an increased risk of sudden cardiac death due to an arrhythmia. Determining a patient’s risk is done by taking very thorough personal and family history, performing a physical examination, and completing a variety of non-invasive testing. This includes cardiac imaging and heart rhythm monitoring.
Using this information, we are then able to identify those patients who are at increased risk and discuss the implantation of a cardioverter-defibrillator (ICD) to both sense and terminate such arrhythmias.
Genetic testing can be used to identify genetic mutations causing HCM so that other family members can also be tested in order to determine their risk of acquiring the disease.
Vigorous exercise in some patients can increase the risk of arrhythmias and fainting episodes. Cardiologists who specialize in HCM will be able to provide individual recommendations on what activities and exercises are safe and not safe.
Amyloidosis is a rare disease that occurs when an abnormal protein called amyloid deposits on organs in the body, including the heart, kidney, liver, gastrointestinal tract, and nervous system.
There are different types of amyloidosis, but there are two major types that account for the majority of cases:
- Immunoglobulin light chain amyloidosis (AL), is the most common form and occurs when excess bone marrow plasma cells secrete the protein that forms amyloid. This form of amyloidosis is similar to multiple myeloma, and most commonly affects the heart, kidneys, and nervous system
- TTR (transthyretin) amyloidosis can occur either from an inherited mutation in the transthyretin gene (ATTR-mutated) or from normal transthyretin (ATTR wild-type).
Diagnosing amyloidosis can be challenging, as symptoms may be vague and similar to those of other common diseases. Amyloidosis can affect nearly every organ system, but most commonly affects the heart, kidney, and nervous system. Symptoms may include fatigue, dizziness, shortness of breath, swelling in the ankles, and tingling of the hands and feet.
Treating amyloidosis depends greatly on the type of amyloidosis and extent of organ involvement. AL amyloidosis is frequently treated with chemotherapy or autologous stem cell transplant. ATTR amyloidosis is treated with medications and, in select cases, cardiac transplant.