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Literature Updates 

ATTR-CM Disease Overview
  1. ATTR-CM is a fatal, underrecognized, and underdiagnosed type of cardiomyopathy.
  2. Patients can present with a wide range of signs and symptoms, both cardiac and extracardiac.
  3. There are 2 approaches to definitively diagnose ATTR-CM after ruling out AL with serum and urine testing: cardiac biopsy and nuclear scintigraphy.
  4. In those cases where results from nuclear scintigraphy are inconclusive, biopsy can play an important role in making an accurate diagnosis.
  5. Genetic counseling and TTR gene sequencing are recommended in all cases of confirmed ATTR-CM.
Latest publications
International guidelines
Diagnosis and treatment of cardiac amyloidosis: a position statement of the ESCWorking Group on Myocardial and Pericardial Diseases

doi:10.1093/eurheartj/ehab072

2021
2021 Advocacy Statements for the Role of 99mTc-Pyrophosphate Scintigraphy in the Diagnosis of Transthyretin Cardiac Amyloidosis: A Report of the Taiwan Society of Cardiology and the Society of Nuclear Medicine of the Republic of China

doi: 10.6515/ACS.202105_37(3).20210420A

2021
ATTR amyloidosis during the COVID 19 pandemic: insights from a global medical roundtable

https://doi.org/10.1186/s13023-021-01834-0

2021
Canadian Cardiovascular Society/Canadian Heart Failure Society Joint Position Statement on the Evaluation and Management of Patients With Cardiac Amyloidosis

https://doi.org/10.1016/j.cjca.2019.12.034

2020
JCS 2020 Guideline on Diagnosis and Treatment of Cardiac Amyloidosis

doi: 10.1253/circj.CJ-20-0110

2020
Diagnosis and treatment of cardiac amyloidosis: position statement of the German Cardiac Society (DGK)

https://doi.org/10.1007/s00392-020-01799-3

2020
Transthyretin amyloidosis: A hidden diagnosis

DOI-10.1097/01.NURSE.0000668628.61785.90

2020
Cardiac Amyloidosis: Evolving Diagnosis and Management A Scientific Statement From the American Heart Association

DOI: 10.1161/CIR.0000000000000792

2020

Prevalence
Prevalence of transthyretin amyloidosis in patients with heart failure and no left ventricular hypertroph

DOI: 10.1002/ehf2.13360

2021
Prevalence of Atrial Fibrillation and Thromboembolic Risk in Wild-Type Transthyretin Amyloid Cardiomyopathy

DOI: 10.1161/CIRCULATIONAHA.120.052136

2021
Prevalence and Outcomes of Concomitant Aortic Stenosis and Cardiac Amyloidosis

https://doi.org/10.1016/j.jacc.2020.11.006

2021
Left Ventricular Amyloid Deposition in Patients with Heart Failure and Preserved Ejection Fraction

doi:10.1016/j.jchf.2013.11.004.

2014
Senile systemic amyloidosis affects 25% of the very aged and associates with genetic variation in alpha2‐macroglobulin and tau: A population‐based autopsy study

DOI: 10.1080/07853890701842988

2008

Diagnosis
ECHO
Value of Longitudinal Strain to Identify Wild-Type Transthyretin Amyloidosis in Patients With Aortic Stenosis

doi: 10.1253/circj.CJ-20-1064

2021
Combining ECG and echocardiography to identify transthyretin cardiac amyloidosis in heart failure

doi:10.1111/CPF.12715

2021
Natural History and Disease Progression of Early Cardiac Amyloidosis Evaluated by Echocardiography

https://doi.org/10.1016/j.amjcard.2020.07.050

2020
Echo Parameters for Differential Diagnosis in Cardiac Amyloidosis A Head-to-Head Comparison of Deformation and Nondeformation Parameters

DOI: 10.1161/CIRCIMAGING.116.005588

2017

Diagnosis
cMRI
Diagnostic value of cardiovascular magnetic resonance in comparison to endomyocardial biopsy in cardiac amyloidosis: a multi centre study

https://doi.org/10.1007/s00392-020-01771-1

2020
CMR Imaging With Rapid Visual T1 Assessment Predicts Mortality in Patients Suspected of Cardiac Amyloidosis

http://dx.doi.org/10.1016/j.jcmg.2013.09.019

2014

Diagnosis
Nuclear Imaging
Nuclear Imaging for Cardiac Amyloidosis: Bone Scan, SPECT/CT, and Amyloid-Targeting PET

https://doi.org/10.1007/s13139-020-00681-4

2021
Transthyretin Cardiac Amyloidosis Scintigraphy Using Planar D-SPECT on Dedicated Cardiac CZT Camera

doi:10.1007/s12350-021-02651-5

2021
Diagnosing Transthyretin Cardiac Amyloidosis by Technetium 99m Pyrophosphate

https://doi.org/10.1016/j.jcmg.2020.08.027

2020
Technetium pyrophosphate nuclear scintigraphy for cardiac amyloidosis: Imaging at 1 vs 3 hours and planar vs SPECT/CT

doi:10.1007/s12350-020-02139-8

2020
Comparison of 18F-sodium fluoride positron emission tomography imaging and 99mT cpyrophosphate in cardiac amyloidosis

doi:10.1007/s12350-020-02425-5

2020
Cardiac Scintigraphy With Technetium-99m-Labeled Bone-Seeking Tracers for Suspected Amyloidosis

https://doi.org/10.1016/j.jacc.2020.04.022

2020
Diagnostic performance of imaging investigations in detecting and differentiating cardiac amyloidosis: a systematic review and meta-analysis

DOI: 10.1002/ehf2.12511

2019

Diagnosis
Biopsy
Non-cardiac biopsy sites with high frequency of transthyretin amyloidosis

DOI: 10.1002/ehf2.13130

2020
Distribution of amyloidosis subtypes based on tissue biopsy site − Consecutive analysis of 729 patients at a single amyloidosis center in Japan

DOI: 10.1111/pin.13041

2020
Abdominal fat pad biopsies exhibit good diagnostic accuracy in patients with suspected transthyretin amyloidosis

https://doi.org/10.1186/s13023-020-01565-8

2020

Diagnosis
Others
Utility of Biomarkers in Cardiac Amyloidosis

https://doi.org/10.1016/j.jchf.2020.03.007

2020
Disease-Specific Biomarkers in Transthyretin Cardiac Amyloidosis

https://doi.org/10.1007/s11897-020-00457-z

2020

Prognosis
Causes of Cardiovascular Hospitalization and Death in Patients with Transthyretin Amyloid Cardiomyopathy

https://doi.org/10.1016/j.amjcard.2021.02.035

2021
Impact of Delayed Diagnosis and Misdiagnosis for Patients with Transthyretin Amyloid Cardiomyopathy (ATTR-CM): A Targeted Literature Review

https://doi.org/10.1007/s40119-021-00219-5

2021
Prediction of Medium-Term Mortality in Japanese Patients With Wild-Type Transthyretin Amyloidosis

doi: 10.1253/circrep.CR-20-0031

2020
Best Practices for Prognostic Evaluation of a Patient With Transthyretin Amyloid Cardiomyopathy

https://doi.org/10.1016/j.jaccao.2019.11.006

2019
Cardiac Amyloidosis: Overlooked, Underappreciated, and Treatable
  1. wtATTR cardiac amyloidosis is significantly more prevalent than previously thought.
  2. ATTR cardiac amyloidosis can be diagnosed noninvasively with nuclear scintigraphy in the absence of monoclonal proteins, but AL cardiac amyloidosis still requires a biopsy.
  3. Recommended serologic testing incudes serum and urine protein electrophoresis with immunofixation (SPEP/UPEP with IFE) as well as analysis of kappa and lambda free light chains, NT-proBNP, troponin-T and eGFR to evaluate the patient for AL amylodosis and stage the patient with CA.
  4. Newly available FDA-approved and emerging treatments aimed at either stabilizing ATTR or silencing ATTR production have shown efficacy in transthyretin amyloid polyneuropathy. Studies have shown tafamidis to be effective for ATTR-CA.
Carpal tunnel syndrome in cardiac amyloidosis:
  1. Compared to the general population, the adjusted prevalence of CTS is higher among elderly men with ATTR patients.
  2. CTS is a prognostic marker in ATTR, independently of cardiac involvement and precedes the diagnosis of cardiac amyloidosis by 5-9 years.
  3. The awareness of this association and time delay offers the possibility of an early pre-clinical ATTR cardiac amyloidosis diagnosis.