Amyloid Cardiomyopathy Program
at Newark Beth Israel Medical Center
At the Advanced Heart Failure Treatment and Transplant Center at Newark Beth Israel Medical Center, our expert cardiologists diagnose and treat cardiac amyloidosis and cardiomyopathy using state-of-the-art, minimally invasive medical tools and therapies.
What Is Amyloidosis and Cardiac Amyloidosis?
Amyloidosis is a disease caused by an accumulation of proteins, called amyloid fibrils, in various organs in the body. It can affect many organs in the body, including the heart, liver, kidneys and nervous system. Cardiac amyloidosis is the form of amyloidosis that affects the heart, stiffening the heart tissue and making it more difficult for the muscle to pump blood effectively. Although amyloidosis is considered a rare disease, it is underdiagnosed and patients often visit many doctors before receiving an appropriate diagnosis.
Causes of Cardiac Amyloidosis
Amyloidosis is caused by changes in proteins that make them insoluble, leading them to remain and build up in organs and tissues. Cardiac amyloidosis occurs when amyloid proteins build up in the heart tissue, causing it to become thick and stiff. Cardiac amyloidosis requires expert care because it can lead to life-threatening heart disease. Early diagnosis and intervention can add years to life and help preserve the quality of life of someone affected by the disease.
Types of Cardiac Amyloidosis
There are two types of amyloidosis that most often cause heart problems.
- Transthyretin amyloidosis (ATTR). ATTR occurs when transthyretin proteins made by the liver are altered due to aging (these are called “wild-type”) or genetic mutation (these are considered hereditary). ATTR can attack other body systems besides the heart and is associated with conditions like carpal tunnel syndrome, spinal stenosis, neuropathy, leg swelling, fainting, digestion problems, sexual dysfunction and more.
- Light chain amyloidosis (AL). AL occurs when plasma cells in the bone marrow produce abnormal proteins.
Symptoms of Cardiac Amyloidosis
Amyloidosis is considered a rare disease and the symptoms can be vague and often mimic other conditions. Symptoms may include:
- Swelling of the ankles and legs
- Weight loss
- Shortness of breath
- Neuropathy – numbness or tingling in hands or feet
- Diarrhea or constipation
- Feeling full quickly
- Severe fatigue
- An enlarged tongue
- Easy bruising including purple patches around the eyes
- An irregular heartbeat
Cardiac Amyloidosis Diagnosis
Diagnosing cardiac amyloidosis as early as possible can help prevent further organ damage. Precise diagnosis is important because treatment varies greatly, depending on the specific condition. Doctors will likely with a thorough medical history and physical exam. After that, one or more of the following tests and procedures may be prescribed:
- Blood tests and urinalysis
- Electrocardiogram (EKG/ECG)
- Echocardiogram (ECHO)
- Nuclear scan
- Magnetic resonance imaging (MRI)
- Biopsy
Treatment of Cardiac Amyloidosis
There is no cure for cardiac amyloidosis. But the Advanced Heart Failure Treatment and Transplant Center at Newark Beth Israel Medical Center are available to help manage patient symptoms, limit the production of further amyloid proteins, and support the affected organs. If left untreated, a person with amyloidosis would have a poor prognosis.
Several medications for transthyretin amyloidosis are now available. These medicines work by either “silencing” the TTR gene or by stabilizing the TTR protein.
Chemotherapy can stop the growth of cells that are making the abnormal protein in people with AL amyloidosis.
At the Advanced Heart Failure Treatment and Transplant Center at Newark Beth Israel Medical Center, we offer heart transplantation as an option for patients with end-stage cardiac amyloidosis. We are one of the few programs in the country with outcomes that match those of patients undergoing cardiac transplantation for other reasons.
Medical and Surgical Procedures
A Multidisciplinary Team Ensures Comprehensive Care
The Amyloid Cardiomyopathy Program employs a multidisciplinary approach to this multiorgan disorder with a team consisting of a neurologist, nephrologist, hematologist and pathologist working together. The program is led by Saurabh Kapoor, MD.
Saurabh Kapoor, MD
Director
Advanced Heart Failure Treatment and Transplant Center
Genetic Testing and Counseling for Amyloidosis Patients
Transthyretin amyloidosis (ATTR) can be caused by a genetic mutation, and it is important to consider how this diagnosis could affect families.
At Newark Beth Israel Medical Center, we routinely do genetic testing and counseling so patients’ families may learn more about their risk of developing cardiac amyloidosis.
