Amyloidosis disease occurs when abnormal proteins (amyloid) build up in one or more organs of the body. When amyloid builds up in the heart tissue, it is known as cardiac amyloidosis or amyloid heart disease.
There are several types of amyloidosis that can affect the heart. It is vital that the correct type is identified, as treatment differs depending on the specific form.
The types of amyloidosis that can affect the heart include:
The Brigham and Women’s Hospital and Dana-Farber Cardiac Amyloidosis Program is a unique service that focuses exclusively on this elusive and deadly condition. Our program was established to fill a significant void in the diagnosis and treatment of systemic cardiac amyloidosis, acquiring a better understanding of cardiac amyloidosis to improve care for patients who have the disease.
The cause of cardiac amyloidosis happens when amyloid build up in the heart tissue. The cardiac amyloid proteins replace normal tissue and stiffen the heart muscle, leading to loss of heart function by decreasing the heart's ability to pump fluid and potentially impacting electrical signaling capabilities. Cardiac amyloidosis can be genetic, develop alongside other diseases, or result from an inflammation-causing medical issue.
Risk Factors for Amyloid Heart Disease
Although the root cause of heart amyloidosis is unknown, there are several risk factors, including:
Cardiac amyloidosis symptoms depend on the organ(s) involved and many symptoms are also common for other forms of heart disease.
Patients with cardiac amyloidosis may initially experience the following symptoms:
Symptoms often worsen as the diseases progresses.
Before a patient comes for their first appointment, they will be asked to provide a detailed medical history. This will help the Brigham and Women’s team to care for them more efficiently.
During the evaluation appointment, every patient will receive:
Some patients will receive:
View a series of short echocardiography video clips that reveal typical features of cardiac amyloidosis.
Cardiac amyloidosis treatment for each patient will vary according to the type of amyloidosis they have, how far the disease has progressed and the presence of any underlying conditions.
Treatment for Light-Chain Associated (AL) Amyloidosis
AL treatment has advanced considerably over the last 10 years. Treatment will be administered to stop the production of abnormal light chains caused by plasma cells, end the progression of the disease and improve damaged organs.
Although amyloidosis is not a cancer, chemotherapy or chemotherapy-like drugs are used to destroy the abnormal plasma cells. A common combination is a chemotherapy agent cyclophosphamide, with a therapy called bortezomib (Velcade) that specifically targets the plasma cells with a steroid (dexamethasone). This therapy, given once a week either by tablet or injection under the skin, is often referred to as “CyBorD.”
An antibody therapy, daratumumab, has also proven highly effective. Daratumumab may be incorporated with CyBorD from the beginning of therapy or may be added a month or two later. Daratumumab is usually given once a week and is generally well-tolerated.
In general, treatment for AL amyloidosis lasts for 6 to 12 months, but some patients may get ongoing maintenance therapy in the form of pills. Your care team will provide more information on the recommended therapy and what to expect from treatment.
Treatment for Transthyretin-Related Amyloidosis (ATTR)
ATTR treatment can include drugs, therapies and cardiac electrophysiology procedures. When all other measures have proven ineffective, physicians may recommend heart transplantation.
Common ATTR amyloidosis treatment options include:
Implantable cardioverter defibrillator or a pacemaker may be recommended to treat atrial and ventricular arrhythmias, which sometimes develop as ATTR progresses.The Cardiac Amyloidosis Program coordinates care with non-cardiac specialists for patients with co-existing conditions such as pulmonary hypertension, peripheral and autonomic neuropathy and renal and gastroenterological disorders.
Treatment for Complications of Amyloid Heart Disease
The Brigham and Women’s team works to ease any complications associated with cardiac amyloidosis. A few areas that are closely monitored include:
Lifestyle changes can also help limit risk factors, including:
Due to innovation in treatment, the prognosis for cardiac amyloidosis has improved and patients often live for multiple years following a diagnosis. Without treatment, cardiac amyloidosis commonly results in death within a year.
Brigham and Women’s Hospital is the site of new discoveries and ongoing research in cardiac amyloidosis, including the use of new medications and highly sensitive echocardiographic techniques for distinguishing cardiac amyloidosis from other heart diseases. Learn more about our research and clinical trials.
The Heart & Vascular Center is located in the Shapiro Cardiovascular Center, across the street from Brigham and Women’s main 75 Francis Street entrance. The Heart & Vascular Center brings together the full range of services in one location, fostering seamless and coordinated care for all cardiovascular patients.
The day of surgery, you care will be provided by surgeons, anesthesiologists and nurses who specialize in surgery for patients with amyloidosis. After surgery, you will go to the post-surgical care unit where you will receive comprehensive care by an experienced surgical and nursing staff.
During your surgery, family and friends can wait in the Shapiro Family Center. Staff members will provide surgery updates and caregivers who leave the hospital will be contacted by cell phone.
Led by Rodney H. Falk, MD, widely recognized as a cardiac amyloidosis expert, our team includes specialists in cardiovascular medicine, cardiac pathology, cardiac imaging, cardiac surgery, gastroenterology, hematology, nephrology, neurology and cardiac research – all Harvard Medical School faculty with extensive experience in cardiac amyloidosis. We work closely with patients and referring physicians, providing the highest standard of medical and supportive care, combined with cutting-edge research and clinical trials.
For over a century, a leader in patient care, medical education and research, with expertise in virtually every specialty of medicine and surgery.