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Question
Hello doctor,
The doctor told me what is hereditary amyloid cardiomyopathy related to transthyretin? What is the method of detecting and treating this disease? I thank the doctor more.
Ngo Cong Huan (2002)
Answer
Answered by Master, Doctor Cao Thanh Tam - Cardiologist - Cardiovascular Center, Vinmec Central Park International General Hospital.
Hi,
To the question “What is transthyretin-associated hereditary amyloid cardiomyopathy?” , the doctor would like to answer as follows:
Amyloidosis (Amyloidosis) is a rare disease that occurs due to the accumulation in the extracellular space of a substance called amyloid. Amyloid is an abnormal protein, synthesized in the bone marrow, that can accumulate locally, cause relatively few symptoms, or be widespread, involve multiple organs, and cause severe multi-organ failure important.
Amyloidosis can occur primary or secondary to a variety of infectious, inflammatory, or malignancies. Diagnosis by biopsy of affected tissues; Amyloidogenic proteins are classified using a variety of immunological and biochemical techniques. Multiple treatments for many types of amyloidosis.
Transthyretin-associated hereditary amyloid cardiomyopathy is a restrictive cardiomyopathy caused by amyloid deposition in the heart.
Diagnosis of cardiac amyloidosis is based on:
Clinical picture, electrocardiogram and echocardiogram Cardiac magnetic resonance imaging Gold standard is myocardial biopsy In addition, it is necessary to do systemic amyloidosis tests such as: Immunoelectrophoresis fluid, Biopsy of abdominal fat, tongue,...
About treatment: Need to coordinate
Supportive care Specific treatment according to amyloid type Currently, there are many specific treatment methods for most forms of amyloid, Although some therapies are still under investigation. For all forms of systemic amyloid, supportive care measures can help relieve symptoms and improve quality of life.
Cardiac magnetic resonance imaging is the most modern method of diagnosis and monitoring of cardiovascular diseases today. Thanks to this technique, doctors can predict and provide effective treatment for congenital heart patients, thereby preventing the progression of the disease, improving the chances of curing the disease or prolonging the life of the patient. sick. Currently, Vinmec International General Hospital is equipped with a 640-slice CT scanner TSX-301C manufactured by Toshiba that is capable of supporting diagnosis on an area up to 16cm wide, with a fast speed allowing one rotation to capture the entire image. heart system, helping to optimally diagnose coronary, vascular and whole body. In particular, this machine can reduce up to 90% of radiation dose, so it can be used for pregnant women when indicated.
If you still have questions about transthyretin-related hereditary amyloid cardiomyopathy, you can go to a Vinmec Health System hospital for further examination and advice. Thank you for trusting and sending questions to Vinmec. Wish you a lot of health.
Best regards!
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