Reverse cardiac remodelling and dysfunction in A97S transthyretin cardiac amyloidosis after tafamidis treatment

Article Cardiac & Cardiovascular Systems

Tafamidis treatment delays structural and functional changes of the left ventricle in patients with transthyretin amyloid cardiomyopathy

Rene Rettl et al.

Summary: Tafamidis can delay the progression of myocardial amyloidosis in ATTR-CM patients, providing structural, functional, and clinical benefits. Cardiac magnetic resonance imaging, including measurement of ECV, may be appropriate for disease-specific therapy monitoring.

EUROPEAN HEART JOURNAL-CARDIOVASCULAR IMAGING (2022)

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Article Geriatrics & Gerontology

Unique Phenotypes With Corresponding Pathology in Late-Onset Hereditary Transthyretin Amyloidosis of A97S vs. V30M

Hsueh-Wen Hsueh et al.

Summary: The study compares the clinical manifestations of A97S and V30M in hereditary transthyretin amyloidosis (ATTRv) and observes differences between the two genotypes. Dysphagia and carpal tunnel syndrome are common in A97S, and the onset age is younger. Autopsy findings in A97S reveal extensive amyloid deposits in the viscera and nerves of the tongue, larynx, and esophagus.

FRONTIERS IN AGING NEUROSCIENCE (2022)

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Article Cardiac & Cardiovascular Systems

Effect of tafamidis on global longitudinal strain and myocardial work in transthyretin cardiac amyloidosis

Gerard T. Giblin et al.

Summary: In patients with ATTR-CM, treatment with tafamidis resulted in less deterioration in GLS, myocardial work index, and efficiency over a 12-month period compared to untreated patients.

EUROPEAN HEART JOURNAL-CARDIOVASCULAR IMAGING (2022)

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Letter Cardiac & Cardiovascular Systems

Response by Elliott et al to Letter Regarding Article, Long-Term Survival With Tafamidis in Patients With Transthyretin Amyloid Cardiomyopathy

Perry Elliott et al.

CIRCULATION-HEART FAILURE (2022)

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Article Cardiac & Cardiovascular Systems

Reduction in CMR Derived Extracellular Volume With Patisiran Indicates Cardiac Amyloid Regression

Marianna Fontana et al.

Summary: The study found that treatment with Patisiran led to reductions in extracellular volume, decreases in N-terminal pro-B-type natriuretic peptide concentrations, increases in 6-minute walk test distances, and evidence of regression in cardiac amyloid in some patients.

JACC-CARDIOVASCULAR IMAGING (2021)

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Review Cardiac & Cardiovascular Systems