Diagnosis of Amyloidosis: A Survey of Current Awareness and Clinical Challenges Among Cardiologists in Switzerland

ConclusionsSurvey results highlight that cardiologists in Switzerland would benefit from information on the latest advances in ATTR to support them in screening heart failure patients and ensure that patients benefit from treatment advances.
Source: Cardiology and Therapy - Category: Cardiology Source Type: research

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AbstractPurpose of reviewTransthyretin amyloid cardiomyopathy (ATTR-CM) is a disease with high morbidity and mortality. This disease is significantly underdiagnosed and is more common than previously appreciated, particularly among older adults and people of African descent. This review discusses recent advances in the diagnosis and treatment for ATTR-CM.Recent findingsHistorically, ATTR-CM was diagnosed via endomyocardial biopsy, a resource-intensive and invasive approach. However, in most cases, ATTR-CM can now be diagnosed non-invasively using bone tracer cardiac scintigraphy, which may facilitate earlier diagnosis. In ...
Source: Current Treatment Options in Cardiovascular Medicine - Category: Cardiology Source Type: research
ConclusionAlthough protein-independent binding is supported by our results, the diagnostic yield of PET was low. We demonstrate here for the first time the low sensitivity of PET for CA.
Source: Journal of Nuclear Cardiology - Category: Nuclear Medicine Source Type: research
We present not ‐yet‐seen multimodal images of a 55‐year‐old female patient with isolated atrial amyloidosis (IAA) who clinically suffered from multiple atrial arrhythmias and heart failure symptoms with preserved left ventricular ejection fraction. We aim to show structural and functional abnormalities det ected by electrophysiological voltage mapping, cardiac magnetic resonance imaging (MRI) [cMRI; atrial strain measurements, late gadolinium enhancement (LGE) visualization], and99mTc ‐DPD scintigraphy. Bipolar voltage mapping performed during two electrophysiological procedures showed diffuse left atrial low‐v...
Source: ESC Heart Failure - Category: Cardiology Authors: Tags: Case Report Source Type: research
AbstractBackgroundIn amyloid patients, cardiac involvement dramatically worsens functional capacity and prognosis.PurposeWe sought to study how the cardio ‐pulmonary exercise test (CPET) could help in functional assessment and risk stratification of patients with cardiac amyloidosis (CA).MethodsWe carried out a multicenter study including patients with light chain (AL) or transthyretin (TTR) CA. All patients underwent exhaustive examination including CPET and follow ‐up. The primary prognostic endpoint was the occurrence of death or heart failure (HF) hospitalization.ResultsWe included 150 patients: 91 AL and 59 TTR CA...
Source: European Journal of Heart Failure - Category: Cardiology Authors: Tags: Research Article Source Type: research
Heart failure due to cardiomyopathy is a frequent manifestation of hereditary transthyretin amyloidosis (hATTR), a progressive, multisystem, and fatal disease that results from the deposition of misfolded transthyretin (TTR) protein in major organs and systems. Other common symptoms are due to polyneuropathy and carpal tunnel syndrome. The hATTR Compass Program offers confidential genetic testing to patients in the United States, Canada, and Puerto Rico suspected of having hATTR with polyneuropathy or with a family history of hATTR.
Source: Journal of Cardiac Failure - Category: Cardiology Authors: Tags: 237 Source Type: research
The objective of our study is to identify the characteristics of patients with this diagnosis in our region.
Source: Journal of Cardiac Failure - Category: Cardiology Authors: Tags: LBCT-015 Source Type: research
Transthyretin amyloidosis (ATTR) is a progressive, fatal disease caused by the formation of amyloid fibrils that misfold and accumulate in various tissue in the body, including the heart, leading to a form of heart failure called transthyretin amyloidosis cardiomyopathy (ATTR-CM). ATTR-CM consists of two subgroups: a wild-type (ATTR-wt) or an inherited mutant autosomal dominant gene (hATTR). ATTR-wt and hATTR have an estimated median survival 3.6 years and 2.5 years after diagnosis, respectively.
Source: Journal of Cardiac Failure - Category: Cardiology Authors: Tags: 145 Source Type: research
Restrictive cardiomyopathy in cardiac amyloidosis (CA) mainly occurs due to light chain (AL) or transthyretin (ATTR) protein deposition in the myocardium. Recurrent congestion related to HF can be challenging to manage in CA, often requiring high dose diuretics and frequent hospitalizations. Though therapies are available for CA that may prolong survival, these do not reverse the cardiomyopathy that may be present at time of diagnosis. Innovative outpatient strategies are needed to effectively manage HF in patients with CA.
Source: Journal of Cardiac Failure - Category: Cardiology Authors: Tags: 143 Source Type: research
AbstractAimsAmyloid cardiomyopathy is an underappreciated cause of morbidity and mortality. Recent evidence suggests that ATTR wild ‐type cardiomyopathy (ATTRwt‐CM) is probably much more common than widely appreciated. So far, no data are available on comparison of mortality from ATTRwt‐CM and other heart failure aetiologies.Methods and resultsThis was a retrospective, observational, cohort study of 2251 patients and their data collected prospectively from May 2000 to June 2018. Long ‐term mortality was the main outcome measure. Underlying cardiomyopathies were classified as amyloid CM (6.1%) [ATTRwt 3.0%; light‐...
Source: ESC Heart Failure - Category: Cardiology Authors: Tags: Original Research Article Source Type: research
No guidelines exist on the use of guideline-directed medical therapy (GDMT) in the setting of transthyretin cardiac amyloidosis (TTR CA) and HFrEF, and its use remains controversial. There is no survival data demonstrating the effects of beta blockers in patients with TTR CA, and the efficacy of angiotensin converting enzyme inhibitors (ACEIs), angiotensin receptor blockers (ARBs), and angiotensin receptor-neprilysin inhibitors (ARNIs) are uncertain. This observational study describes the outcomes of patients with TTR CA and HFrEF treated with GDMT.
Source: Journal of Cardiac Failure - Category: Cardiology Authors: Tags: 367 Source Type: research
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