Heart Failure

For providers

We offer the expertise in the evaluation, diagnosis and treatment of infiltrative cardiomyopathies, including cardiac amyloidosis. We evaluate patients suspected of having infiltrative cardiomyopathies with a complete array of cardiac imaging tools including transthoracic echocardiography with global longitudinal strain (GLS), cardiac MRI with late gadolinium enhancement (LGE), and technetium Tc-99m pyrophosphate (PYP) scan. Ultimately, in the case of indeterminate findings, we can proceed with invasive cardiac measures, including endomyocardial biopsy if appropriate. Our staff includes a cardiac amyloidosis coordinator that will arrange the collection of patients’ medical records prior to a consult visit, meet with the patient to discuss the treatment plan delineated by the provider, facilitate the insurance approval process of ATTR amyloidosis specific drug therapies, along with monitoring the response to treatment and follow-up cardiac studies.

In the case an AL amyloidosis diagnosis is made, we work closely with our colleagues from the oncology and hematology group at Utica Park Clinic to determine treatment or to vet any overlapping features consistent with monoclonal gammopathy of undetermined significance (MGUS).

For patients

The Advanced Heart Failure Center at OHI offers the expertise in the evaluation, diagnosis, and treatment of infiltrative cardiomyopathies, including cardiac amyloidosis (am-uh-loi-DO-sis).

Cardiac amyloidosis is a rare disease that occurs when either bone marrow or liver produces an abnormal protein called amyloid. This protein can cause problems as it builds up in various organs and soft tissues, including the heart, liver, kidneys, spleen, digestive tract, and nervous system.

There are different types of amyloidosis and symptoms can vary, depending on the affected organ or soft tissue. Amyloidosis is not curable, but its progression can be interrupted and, at times, reversed with specific therapies.

Common types of amyloidosis:

AL amyloidosis (Light Chain): The most common type of Amyloidosis, caused by a bone marrow disorder. It is often treated with chemotherapy or stem cell transplant. We work closely with our hematology/oncology colleagues, who determine the best treatment plan for these patients.

Hereditary amyloidosis (hATTR): Caused by a gene mutation and involves an amyloid protein produced in the liver. There are specific therapies that stabilize this protein or halt its production, preventing further damage and progression of the disease. These infusions and injections can be prescribed and administered in our center.

Wild-type ATTR amyloidosis: Caused by deposition of an otherwise normal amyloid protein over time, related to aging. This type can ultimately lead to heart failure. An oral regimen is the most common treatment.

Common signs and symptoms:

• Bilateral carpal tunnel syndrome
• Chronic gastrointestinal issues such as diarrhea
• Bicep tendon rupture
• Dizziness
• Fatigue
• Heart failure symptoms, such as shortness of breath and swelling in hands, arms, legs and feet
• Muscle weakness

Amyloidosis screening and diagnosis

Diagnosis can be a lengthy process. Symptoms vary and affect people differently. That is why it is important to see a doctor who has experience diagnosing and treating amyloidosis. Your signs and symptoms will determine what tests are ordered for you.

• Autonomic testing, including functions like heart rate, body temperature, breathing rate and digestion
• Biopsy of the skin, nerves, muscle, heart, liver or abdominal fat pad, the examination of a sample of tissue under a microscope
• Blood tests including genetic testing
• Cardiac MRI
• Echocardiography - an ultrasound of the heart
• Technetium Tc-99m pyrophosphate (PYP) scan of the heart

For patients

Cardiopulmonary exercise testing (CPX) help physicians understand how well your lungs, heart and skeletal muscles work together to process oxygen. This test may be recommended if you have difficulty catching your breath or find a need to rest more often while doing daily activities. If you have heart failure, this is a helpful tool to help your doctor understand the severity of the condition and will help in planning future care.

Your ability to perform daily activities without shortness of breath, dizziness or extreme fatigue depends on three things. The CPX test checks all three.

1. How well you bring air into your lungs?
2. How well does your heart pump oxygen rich blood out to the body?
3. liHow well do the factories in your muscles process oxygen to allow the muscles to work?

What to expect:

• You will be hooked up to an EKG to monitor your heart rate and rhythm using sticky pads that will be placed on your skin under your clothing.
• A blood pressure cuff will be put on your arm and a blood oxygen sensor placed on your finger.
• While seated, you will be asked to blow in and out of a special sensor that measures your ability to bring air in and out of your lungs. During the exercise part of the test, the air you breathe in and out will be measured using either a comfortable face mask or mouthpiece.
• Then you will be set up on either a treadmill or recumbent bike for exercise. You’ll start walking slowly on the treadmill or pedaling on a recumbent bike without any resistance to get your body ready for exercise. As the test progresses, the speed and elevation of the treadmill, or resistance to pedaling the bike will increase. The test is specifically staged to your ability, and you may stop the test at any time. The goal is to exercise as hard as possible since that will provide the most information.
• While testing your blood pressure and blood oxygen level, perceived exertion and any symptoms will be monitored.
• The data collected will be interpreted and reviewed with you by your physician.

How to prepare for CPX testing:

• Wear comfortable exercise clothing and shoes for this test. Do not wear sandals.
• Avoid putting lotion or powder on your chest on the day of the test.
• Do not take your water pill the morning of the test but take all other medications as they are prescribed.
• Eat a light meal at least an hour before the test. An example would be a piece of toast and fruit or half a sandwich and piece of fruit. Please do not eat a large meal prior to testing as you will be exercising.
• If you use a rescue inhaler when you have shortness of breath, bring it with you.
• You may be tired after completing the test. Consider having someone else drive you to the appointment.

For providers

CPX is an important clinical tool used in the evaluation of undiagnosed exercise intolerance and for objective determination of functional capacity and impairment. In the setting of heart failure and other cardiac conditions, CPX is a valuable tool for staging and surveillance and an important step in the identification of patients in need of advanced modalities such as mechanical circulation and/or transplant.

Peak exercise capacity is defined as the maximum ability of the cardiovascular system to deliver oxygen to exercising skeletal muscle and the ability of that muscle to extract oxygen from the blood. Consequently, exercise tolerance is determined by three factors: pulmonary gas exchange, cardiovascular performance (including the peripheral vascular tree) and skeletal muscle metabolism.

CPX provides objective assessment of integrative exercise responses involving the pulmonary, cardiovascular and skeletal muscle systems not reflected in other measurements of individual organ system function. During testing, respiratory oxygen uptake (VO2), carbon dioxide production (VCO2) and ventilatory efficiency are measured during a symptom-limited exercise test.

Specific features of testing include select pulmonary function tests to identify potential obstructive ventilatory processes and maximal exercise performance utilizing either a treadmill or recumbent bicycle ergometer. Patients receive ongoing monitoring of EKG, pulse oximetry, blood pressure and cardiovascular and nervous system symptoms during testing.

Patients who are dependent on supplemental oxygen, continuous inotrope infusion, or unable to exercise on a treadmill or bicycle are not candidates for CPX testing.

CPX data is interpreted and reported by a specially trained cardiologist.

For patients

As a part of your treatment, your doctor and health care providers may consider aquapheresis, also known as ultrafiltration therapy. This therapy safely and effectively removes excess salt and water from patients who are suffering from fluid overload due to heart failure.

Your health care provider may prescribe a low-salt diet, fluid restriction or other therapies to help reduce fluid – such as a water pill or diuretic, given orally or intravenously. However, these treatments do not always work effectively, resulting in a need for alternative therapy – such as aquapheresis.

For providers

Aquapheresis (ultrafiltration) is a process of fluid removal when a patient is diuretic resistant. A patient is considered diuretic resistant when:

• Patient is > 10 pounds over dry weight
• Previous hospitalizations with ineffective diuretic effect
• Patient cannot achieve a goal of -2 liters at 24 hours
• No significant difference in patient’s global assessment of symptoms in 24 hours.

Goals of aqupaheresis:

• Reduction in hospital readmission: prevent patients from being discharged when they are “wet” (volume overloaded)
• Reduction of length of stay if aqupheresis is started early (< 24 hours of admission)
• Stable renal function

Monitor BMP/CMP prior to starting aqupheresis can reduce rate of fluid removal as needed. This is usually managed by Advanced Heart Failure team in units with specially trained nursing staff (2 heart and 3 heart)