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Cardiovascular disease that can lead to death; tends to be ‘hereditary’!

Maintaining a healthy lifestyle is of utmost significance, but there are many things people may do to improve their heart health. Along with that, there has to be a lot of knowledge on how to maintain the heart operating in a healthy way and the warning signs and symptoms to look out for.

It is important for people to be aware that an uncommon condition termed ‘amyloid cardiomyopathy,’ sometimes known as ‘cardiac amyloidosis,’ can be fatal. According to Dr. Jamshed Dalal, director of cardiac sciences at Kokilaben Dhirubhai Ambani Hospital in Mumbai, ‘it is a disorder where faulty proteins accumulate in the heart; heart failure comes from its inability to function properly owing to this protein build-up’.

Cardiac amyloidosis can be caused by a number of different things, says the doctor. Some people are born with it, while others may get it independently or as a result of another illness. Some people inherit it from their parents. Though the majority of its types may be controlled and treated, cardiac amyloidosis cannot be healed.

Two categories of cardiomyopathy

1. Dilated cardiomyopathy, which causes the heart’s blood pumping chambers to weaken, expand, and dilate, resulting in poor heart pumping.

2. Hypertrophic cardiomyopathy, in which the heart muscle swells, tightens, and loses some of its capacity to accept blood. Amyloidosis is the cause of a highly particular kind of hypertrophic cardiomyopathy. Transthyretin amyloid cardiomyopathy (ATTR-CM), according to Dr. Dalal, is a ‘rare but severe example of transthyretin fibrils piling up in the heart muscles,’¬†which can cause hypertrophic or restrictive cardiomyopathy.

‘Transthyretin is a particular protein that circulates vitamin A and thyroxine hormone in the body, which are vital for maintaining muscle function and other systems. Transthyretin amyloid cardiomyopathy can cause the heart’s pace and rhythm to change, as well as cause new or worsening heart failure’, according to the expert. This makes early diagnosis of it crucial.

The expert explains that while most cardiologists are aware of amyloid cardiomyopathy, transthyretin amyloid cardiomyopathy detection is frequently overlooked because of its rarity and diagnostic challenges. ‘ Amyloid cardiomyopathy affects 15% of individuals with hypertrophic cardiomyopathy. Physicians must now be on the lookout for transthyretin amyloid cardiomyopathy’. Dr. Dalal warns that this uncommon cardiac condition is frequently associated to young individuals and those who have carpal tunnel syndrome.

How is it discovered?
He claims that ‘ECG frequently provides a hint, and 2D echocardiography reveals characteristics of hypertrophic cardiomyopathy; cardiac MRI sheds more information’.¬†However, the most reliable test for identifying this uncommon type of cardiac amyloidosis is the pyrophosphate (PYP) nuclear scan. A special camera picks up the radioactive tracers that provide a 3D picture of the heart anatomy during this test, which is accessible in many hospitals and lasts for about an hour or two.

‘Without early detection and treatment, irreversible damage is possible and people often die within three years of the disease. Treatment of cardiac amyloidosis requires a multidisciplinary approach with a specialised inter-professional team of experts in cardiology, hematology, radiology and nuclear medicine,’ the doctor concludes.



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