Cardiac Amyloidosis: A New Heart for Frank

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PHILADELPHIA, Pa. (Ivanhoe Newswire) — Cardiac amyloidosis, also called stiff heart, occurs when abnormal proteins build up and settle in body tissues. If these amyloid proteins travel to the heart, it can be deadly, and sometimes the only option for survival is a heart transplant.

Sixty-eight-year-old Frank Tummarello is back to enjoying quiet moments with his wife, following a ten-year battle with a deadly disease. Although he was initially diagnosed with atrial fibrillation, doctors suspected something more threatening: cardiac amyloidosis.

“I didn’t even know what it was,” Tummarello admitted to Ivanhoe.

Frank’s liver produced too much amyloid protein. It deposited in his heart, enlarging and thickening the muscle and preventing proper blood flow throughout his body.  Soon, this previously active father and husband found it difficult to function.

Rene Alvarez, MD, Vice Chief, Section of Cardiology at Temple University Hospital explained, “It was very difficult for him to have any quality of life. He was in and out of the hospital, very short of breath, couldn’t walk 50 feet. So the prognosis of those patients once they develop heart failure from cardiac amyloid is very, very poor. The one-year survival is at best, 30 to 40 percent in those patients.” (Read Full Interview)

The only option left for Frank was a transplant.

“I just wanted to get away, get rid of the old feeling. This heart would give me the opportunity to get rid of it,” Tummarello said.

The surgery took place in September of 2016.  It made a dramatic difference.

Dr. Alvarez continued, “He knew he had a death sentence, that he was dying, and he got the gift of life.  And the gift of life was a heart transplant.”

“The whole Temple team from Dr. Alvarez told me you’re gonna feel like a different person when this is all over and guess what; they’re absolutely right,” Tummarello stated.

Symptoms of cardiac amyloidosis include fatigue, heart palpitations, shortness of breath and swelling of legs and ankles. About 4,500 people a year are diagnosed with the condition.

Contributors to this news report include: Cyndy McGrath, Supervising Producer; Donna Parker, Field Producer; Kirk Manson, Videographer; Gabriella Battistiol, Assistant Producer; Roque Correa, Editor.

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MEDICAL BREAKTHROUGHS

RESEARCH SUMMARY

TOPIC:            CARDIAC AMYLOIDOSIS: A NEW HEART FOR FRANK

REPORT:       MB #4289

BACKGROUND: Cardiac Amyloidosis is caused by the deposits of protein (amyloid) in the heart tissue. Over time, the proteins will replace the normal tissue which leads to damaged heart signals (heart block) and also affects the way the electric signals move through the heart.. When symptoms do occur they may be excessive urination at night, fatigue, palpitations, shortness of breath, swelling of parts of the body, and trouble breathing while lying down.
(Source: https://medlineplus.gov/ency/article/000193.htm )

TREATMENT/TESTS:  There are numerous tests needed to diagnose cardiac amyloidosis.  There is the chest or abdomen CT scan, which is the “gold standard” for diagnosing this disorder. There is also the coronary angiography, which uses a special dye and x-rays to see how blood flows through arteries in the heart. Additionally, the electrocardiogram (ECG), echocardiogram, magnetic resonance imaging (MRI), nuclear heart scans (MUGA, RNV), and post emission tomography (PET), are all tests that help to diagnose cardiac amyloidosis. Heart transplant is an option for patients with this disorder. It is also important to limit fluid intake, as the heart becomes too stiff to pump blood and can lead to fluid build-up, causing leg swelling and breathlessness.  Diuretics may be prescribed to help the body lose excess salt and water to prevent ankle swelling and breathlessness. Calcium-channel blockers and beta-blockers may be used with patients who have atrial fibrillation.

(Sources: https://medlineplus.gov/ency/article/000193.htm )

http://www.amyloidosis.org.uk/cardiac-amyloidosis-2/treatmetn-of-cardiac-amyloidosis/)

NEW RESEARCH: With chemotherapy options expanding over the years, prognosis for this disorder has greatly improved. In fact, there is a first-line treatment of proteasome inhibitor bortezomib, plus dexamethasone and an alkylating agent (BDex+AA) improves survival in patients with heart failure due to cardiac amyloidosis.  As no U.S. Food and Drug Administration-approved drugs are currently available for the treatment of HFPEF or TTR cardiac amyloidosis, the development of medications that prevent TTR-mediated organ toxicity has emerged as an important therapeutic goal.

(Source: http://www.acc.org/latest-in-cardiology/articles/2015/10/13/08/35/emerging-therapies-for-transthyretin-cardiac-amyloidosis )

FOR MORE INFORMATION ON THIS REPORT, PLEASE CONTACT:

Jennifer Lee

Jennifer.Lee3@tuhs.temple.edu

If this story or any other Ivanhoe story has impacted your life or prompted you or someone you know to seek or change treatments, please let us know by contacting Marjorie Bekaert Thomas at mthomas@ivanhoe.com

Doctor Q and A

Read the entire Doctor Q&A for Rene Alvarez, MD

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