Cardiac amyloidosis (CA) is an under-diagnosed disease presenting as a restrictive cardiomyopathy with high morbidity and mortality. Wild-type transthyretin amyloid cardiomyopathy (ATTR-CM) is mostly seen in el
Types of Cardiac Amyloidosis (CA) Over 30 proteins can form amyloid deposits in humans, however only about 10 cause cardiac disease (Table 2). The vast majority of CA are caused by either ATTR or AL deposition. Table 3 summarises the types of cardiac involvement. Table 2. Proteins in amy...
Once considered a rare disease, cardiac amyloidosis is now recognized as a significant cause of heart failure. Breakthroughs in noninvasive imaging techniques for ATTR-CM have reshaped our diagnosis of the disease, and there is growing evidence to suggest it is more prevalent than previously believe...
The most common type of cardiac amyloidosis is transthyretin amyloidosis (ATTR-CM). Early forms of the disease can often go undetected. Effective pharmacological treatments are available for ATTR-CM. However, current treatment options may be more effective when used earlier in the disease, making ea...
(LV) hypertrophy with unidentified underlying mechanisms, precision-based therapies include genetic evaluation for hypertrophic cardiomyopathy, Fabry disease, Noonan syndrome, or other genetic alterations of LV hypertrophy. Meanwhile, evaluation for possible cardiac amyloidosis by imaging modalities is also ...
Conclusion: Amyloidosis is relatively fatal disease. Patients often have a short life expectancy after symptom onset. Early diagnosis is crucial to proper management and better prognosis.Binay Kumar AdhikariYonggang WangBo LiQuan LiuWeihua Zhang心血管病(英文)...
Sudden cardiac death (SCD) is not uncommon in immunoglobulin light-chain amyloidosis (AL) and has been usually attributed to pulseless electrical activity (PEA) or agonal bradycardia occurring in the late stages of the cardiomyopathy. In addition, the historically reported survival of <12 months in...
Cardiac amyloidosis has traditionally been associated with a poor prognosis, with a median survival of less than 1 year once patients present with heart failure. As consensus guidelines recommend against implantable cardioverter-defibrillator (ICD) placement for the primary prevention of sudden cardiac ...
Induced pluripotent stem cells: applications in regenerative medicine, disease modeling, and drug discovery. Front Cell Dev Biol. 2015;3:2. Article PubMed PubMed Central Google Scholar Agrawal T, Nagueh SF. Echocardiographic assessment of cardiac amyloidosis. Heart Fail Rev. 2022;275:1505–13. ...
However, if the clinical context is not taken into consideration, the risk of misdiagnosis may be high (e.g., sarcomeric HCM, cardiac amyloidosis or even Anderson-Fabry disease). mtDNA-related diseases are potentially lethal, and death most commonly occurs due to recurrent stroke-like episodes,...