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 elderly patients, with increasing prevalence as life expectancy is growing. New diagnostic ...
The current state of amyloidosis My loving grandfather's recent diagnosis of Amyloidosis, a disease with an unknown cause, few experimental treatment options, and no cure, inspired this ar... N Sheppe 被引量: 2发表: 2004年 The current state of amyloidosis therapeutics and the potential role of...
Amyloidosis inserts abnormal proteins into tissues – in the heart, that results in an insidious decline cardiac function marked by increased stiffness (requiring high filling pressures that wet the lungs) and decreased contractility or inotropy (pumping ability). resulting in poor circulation of nutrien...
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
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,...
Mounting evidence has shown that LV GLS is a very powerful prognostic predictor, not only for patients with cardiac disease but also for those with systemic diseases, such as hypertension12, septic shock14, and chronic kidney disease11,13. Here, we demonstrated the prognostic value of LV GLS ...
16 Increased native T1 time is a surrogate marker of myocardial fibrosis in other disease states such as amyloidosis and hypertrophic obstructive cardiomyopathy.17, 18, 19 Although increased myocardial native T1 times have been demonstrated in early CKD,20 until now the assessment of native T1 times...
(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 ...
Previously thought to be a rare disease, ATTRwt cardiac amyloidosis may account, variably, for between 5% and 18%of patients over the age of 65 years with heart failure with preserved ejection fraction (HFpEF).1,2 Furthermore, specific pathogenic mutations in the transthyretin gene are ...
Palladini G, Campana C, Klersy C, Balduini A, Vadacca G, Perfetti V, et al. Serum N-terminal pro-brain natriuretic peptide is a sensitive marker of myocardial dysfunction in AL amyloidosis. Circulation. 2003;107:2440–5. ArticleCAS ...