There are many things that people can do for themselves Heart BrainsAnd maintaining a healthy lifestyle is paramount.
Additionally, there is a need for a great deal of awareness of how the heart functions in a healthy way, and for this, noticeable signs and symptoms may be indicative of health issues.
People should be aware that something called ‘amyloid cardiomyopathy’, often referred to as ‘cardiac amyloidosis’, can be a life-threatening rare disease. “It is a condition where there is a build-up of defective proteins in the heart; the inability to function properly due to this protein build-up results in heart failure,” said Dr Jamshed Dalal, Director, Cardiology, Kokilaben Dhirubhai Ambani Hospital, Mumbai explains.
According to doctors, there are many causes of cardiac amyloidosis. Some people inherit it from their parents, while others can get the disease on their own or as a result of other diseases. “Cardiac amyloidosis cannot be cured; most forms can be managed and treated.”
Doctors tell that there are two types of cardiomyopathy:
1. Dilated CardiomyopathyIn which the blood-pumping chambers of the heart weaken and dilate/enlarge, and the heart does not pump well.
2. Hypertrophic Cardiomyopathy, where the heart muscle thickens, becomes restrictive, and its ability to receive blood is impeded. A very specific type of hypertrophic cardiomyopathy is caused by amyloidosis.
Dalal states that ‘transthyretin amyloid cardiomyopathy’ (ATTR-CM) is “a rare but serious case of a buildup of transthyretin fibrils in the heart muscle”, which can result in hypertrophic/restrictive cardiomyopathy.
“Transthyretin is a specific protein that is needed for vitamin A and thyroxine hormones – muscle control and other systems throughout the body. Transthyretin amyloid cardiomyopathy manifests as a new or worsening stop beatingand it can also affect the electrical system, affecting heart rate and rhythm,” he explains. Therefore, early detection is important.
Without early detection and treatment, irreversible damage is possible and people often die within three years of illness. (Photo: Getty/Thinkstock)
Experts say most cardiologists are aware of amyloid cardiomyopathy, but because of its rarity and diagnostic difficulties, the detection of transthyretin amyloid cardiomyopathy is often missed.
“Among patients with hypertrophic cardiomyopathy, about 15 percent have amyloid cardiomyopathy. It becomes essential for physicians to suspect transthyretin amyloid cardiomyopathy. Young adults and people with carpal tunnel syndrome are often associated with this rare heart disease,” Dr Dalal warns.
How is it detected?
“ECG often gives a clue, and 2D echocardiography indicates features of hypertrophic cardiomyopathy; cardiac MRI sheds further light,” he says.
However, the most commonly used diagnostic test is the pyrophosphate (PYP) nuclear scan, which makes it possible to accurately diagnose this rare form of cardiac amyloidosis. “This test, available in many hospitals, lasts an hour or two, where the drug is administered intravenously and a special camera picks up a radioactive tracer that gives a 3D image of the heart structure.”
“Without early detection and treatment, irreversible damage is possible and people often die within three years of illness. Treatment of cardiac amyloidosis requires a multidisciplinary approach with a specialized inter-professional team of experts from cardiology, hematology, radiology and nuclear medicine,” concluded the doctor.
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