Cardiac amyloidosis is an infiltrative cardiomyopathy caused by the deposition of insoluble amyloid fibrils in the myocardium, leading to abnormal cardiac function and heart failure. Diagnosis is often challenging due to its diverse symptoms and related comorbidities. Although endomyocardial biopsy is the gold standard for diagnosis, a complete diagnostic approach often includes non-invasive methods such as Cardiac Magnetic Resonance (CMR) and nuclear medicine techniques. Nuclear medicine bone-seeking agents are useful for diagnosing the transthyretin (ATTR) amyloidosis subtype. CMR is especially useful when echocardiography results are inconclusive or for differentiating cardiac amyloidosis from other conditions like hypertrophic cardiomyopathy. CMR provides a comprehensive evaluation of morphological and functional abnormalities, inversion time, late gadolinium enhancement, and can include advanced techniques such as T1 mapping and extracellular volume quantification for diagnostic and prognostic purposes. This review outlines how CMR and nuclear medicine are integral to the diagnostic process for cardiac amyloidosis, emphasizing their crucial roles and the synergy between various diagnostic techniques in assessing suspected cases. ESSENTIALS: REQUIRED SUMMARY STATEMENT: Nuclear medicine PYP scans and CMR play a pivotal role in the non-invasive diagnosis of cardiac amyloidosis, alongside other essential diagnostic modalities.
Download full-text PDF |
Source |
---|---|
http://dx.doi.org/10.1016/j.acra.2025.01.022 | DOI Listing |
Cureus
February 2025
Department of Cardiology, National Hospital Organization Hiroshima-Nishi Medical Center, Otake, JPN.
Transthyretin cardiac amyloidosis (ATTR-CA) involves the buildup of transthyretin protein in the heart muscle in the form of amyloid fibrils, which can affect heart structure and function. Common ECG findings of ATTR-CA include low QRS voltage and a pseudo-myocardial infarction (MI) pattern, defined as pathological Q waves or QS complexes in two consecutive leads without a history of MI or echocardiographic evidence of akinetic areas. Here, we present a case of ATTR-CA in a very elderly patient, in whom pathological Q waves on ECG were true indicators of a prior inferior MI.
View Article and Find Full Text PDFNucl Med Commun
March 2025
Department of Nuclear Medicine and Ultrasound.
Objective: This study aimed to assess the diagnostic accuracy of a novel semiautomated method to calculate relative myocardial wall uptake (RCU) of 99mTechnetium-pyrophosphate SPECT/CT in suspected cardiac transthyretin amyloidosis (ATTR).
Methods: Prospective analysis of 108 participants with suspected ATTR. Using novel software, we quantified radiopharmaceutical uptake in the wall of each chamber of the heart at 3 h, calculating RCU for combined ventricular (RCU-V) and whole-heart (RCU-W) measures as a ratio over physiological blood-pool activity.
Arch Cardiovasc Dis
February 2025
Bordeaux University Hospital, 33000 Bordeaux, France; University of Bordeaux, 33000 Bordeaux, France; CIC-P 1401, 33600 Bordeaux-Pessac, France; Inserm 1045, 33600 Pessac, France.
Background: Patients with left ventricular hypertrophy (LVH) often maintain preserved left ventricular ejection fraction in the early stages of the disease. There is a need to identify simple and reliable variables beyond left ventricular ejection fraction to recognize those at risk of developing adverse clinical outcomes.
Aims: To examine left atrial (LA) strain in patients with hypertrophic cardiomyopathy (HCM), cardiac amyloidosis (CA) and Fabry disease (FD), pathologies known to cause LVH, and the relationship between LA strain and adverse clinical outcomes.
Proc Natl Acad Sci U S A
March 2025
Department of Integrative Structural and Computational Biology and Skaggs Institute of Chemical Biology, Scripps Research, La Jolla, CA 92037.
The transthyretin (TTR) tetramer, assembled as a dimer of dimers, transports thyroxine and retinol binding protein in blood plasma and cerebrospinal fluid. Aggregation of wild type (WT) or pathogenic variant TTR leads to transthyretin amyloidosis, which is associated with neurodegenerative and cardiac disease. The trigger for TTR aggregation under physiological conditions is unknown.
View Article and Find Full Text PDFEgypt Heart J
March 2025
Padjadjaran University, Bandung, Indonesia.
Background: Transthyretin cardiac amyloidosis (ATTR-CA) is a progressive cause of diastolic heart failure associated with poor prognosis. Currently available treatment, tafamidis, a TTR stabilizer, is highly effective and tolerable but is not cost-effective. Hence, we aim to evaluate the efficacy and safety of a mechanistically similar but more affordable TTR stabilizer, diflunisal, in patients with ATTR-CA.
View Article and Find Full Text PDFEnter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!