Transthyretin cardiac amyloidosis is prevalent in 16% of patients with severe calcific AS undergoing TAVR and is associated with a severe AS phenotype of low-flow low-gradient with mildly reduced ejection fraction. Average tissue Doppler mitral annular S' of < 6 cm/s may be a sensitive measure that should prompt a confirmatory 99mTc-PYP scan and subsequent testing for ATTR-CA. Prospective assessment of outcomes after TAVR is needed in patients with and without ATTR-CA.
In this multicenter study, Tc 99m PYP cardiac imaging conferred a high level of sensitivity and specificity for differentiation of patients with ATTR cardiac amyloidosis (irrespective of genotype) from patients with AL cardiac amyloidosis and patients with nonamyloid heart failure with preserved ejection fraction. An H/CL ratio of 1.6 or greater was associated with worse survival among patients with ATTR cardiac amyloidosis. Among patients for whom there is a high clinical suspicion of cardiac amyloidosis, Tc 99m PYP may be of diagnostic and prognostic importance.
Advances in diagnostic imaging have increased the recognition of coexisting transthyretin cardiac amyloidosis (ATTR-CA) and severe aortic stenosis (AS), with a reported prevalence between 8-16%. In this prospective study, we aimed to evaluate the implications of ATTR-CA on outcomes after transcatheter aortic valve replacement (TAVR).
Amyloidosis is caused by extracellular deposition of abnormal protein fibrils, resulting in destruction of tissue architecture and impairment of organ function. The most common forms of systemic amyloidosis are light-chain (AL) and transthyretin-related (ATTR). ATTR can result from an autosomal dominant hereditary transmission of mutated genes in the TTR (ATTRm) or from a wild-type form of disease (ATTRwt), previously known as senile cardiac amyloidosis (SCA). With the aging of the worldwide population, ATTRwt will emerge as the most common type of cardiac amyloidosis that clinicians encounter. Diagnosis of systemic amyloidosis is often delayed, either due to the false assumption that it is a rare disease, or due to misdiagnosis as a result of mistaking it with other conditions. Clinicians must integrate clinical clues from history, physical exam, and common diagnostic tests to raise suspicion for ATTRwt. The historical gold standard for diagnosis of cardiac amyloid is endomyocardial biopsy (EMB) with pathological distinction of precursor protein type, but this method often results in delayed diagnosis given the limited availability of expertise to perform and interpret the EMB. Emerging noninvasive imaging modalities provide easier, accurate screening for ATTRwt. These modalities include: advanced echocardiography, using strain imaging and the myocardial contraction fraction; nuclear scintigraphy, which can differentiate between ATTR and AL cardiac amyloid; and cardiac magnetic resonance, using extracellular volume measurement, late gadolinium enhancement, and distinct T1 mapping. These novel approaches reveal insights into the prevalence, clinical course, morphological effects, and prognosis of ATTRwt.
The number of publications in CD is increasing, out of proportion to the overall growth of the peer-reviewed medical literature. CD publications are spread throughout a larger number of journals, but are more dominated by high-volume authors. Economic factors are associated with national contributions to the world literature in CD.
Older age was not independently associated with short-term or longer-term survival among V-A ECMO patients, but may reflect greater comorbidity, suggesting that age alone may not disqualify patients from V-A ECMO therapy.
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