The Imaging Diagnosis of Less Advanced Cases of Cardiac Amyloidosis: The Relative Apical Sparing Pattern

Ono, Katsumichi; Ishimaru, Go; Hayashi, Miho; Bae, Yun‐Jung; Ito, Takashi; Izumo, Toshiyuki; Murata, Ken T.

doi:10.2169/internalmedicine.56.7610

Cited by 7 publications

(5 citation statements)

References 19 publications

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“…Proposed mechanisms include: 1. a reduced infiltration with amyloid fibrils at the apex, compared to basal segments; 2. an increased tendency towards apoptosis at basal level, as a result of the higher parietal stress [25,28,33]. This typical appearance was first described in late-stage CA, but there are studies that suggest its presence in less affected hearts [31,34]. 2D STE is helpful in differentiating the thickness of myocardium caused by amyloidosis from a real left ventricular hypertrophy.…”

Section: Speckle-tracking Echocardiographymentioning

confidence: 94%

Cardiac Amyloidosis

Eötvös¹,

Paștiu²,

Zehan³

et al. 2021

Cardiomyopathy - Disease of the Heart Muscle

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Amyloidosis represents a heterogeneous group of disorders caused by amyloid fibril deposition in the extracellular space in different organs. Among the many types of amyloidosis cardiac involvement occurs almost exclusively with immunoglobulin light chain amyloidosis (AL amyloidosis) or transthyretin amyloidosis (ATTR amyloidosis). When present cardiac amyloidosis (CA) has a significant impact on disease prognosis. The typical clinical presentation in CA is that of a restrictive cardiomyopathy. Clinical suspicion of CA is based on clinical, laboratory and electrocardiographic findings. The diagnosis is confirmed using echocardiography, cardiac magnetic resonance imaging, biopsy, and/or bone scintigraphy. A precise definition of amyloidosis type is essential for choosing the specific treatment for this condition. Treatment of CA has two components: general treatment of congestive HF, and specific treatment of the underlying protein misfolding disorder.

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Section: Speckle-tracking Echocardiographymentioning

confidence: 94%

Cardiac Amyloidosis

Eötvös¹,

Paștiu²,

Zehan³

et al. 2021

Cardiomyopathy - Disease of the Heart Muscle

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“…Este patrón (preservación apical) fue descrito para AC en estadios avanzados, con hipertrofia promedio mayor a 14 mm, pero recientemente se describió para pacientes con menor grado de hipertrofia (24,6) .…”

Section: Deformación Longitudinal Global < -15%unclassified

Aportes del ecocardiograma y las técnicas de deformación miocárdica en la amiloidosis cardíaca. Bases de la técnica y hallazgos de utilidad diagnóstica

2021

Rev.Urug.Cardiol.

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La amiloidosis cardíaca es una miocardiopatía restrictiva infiltrativa secundaria al depósito extracelular de amiloide. Las diferentes técnicas de imagen cardíaca permiten la evaluación de forma no invasiva. El ecocardiograma juega un rol central en la evaluación diagnóstica, pronóstica y ayuda a guiar el tratamiento. El objetivo de este artículo de revisión es describir las diferentes herramientas que nos brinda la ecocardiografía, poniendo énfasis en el strain por speckle tracking y describir su rol en el diagnóstico de esta patología. Palabras clave:amiloidosis cardíaca strain speckle tracking doppler tisular Contributions of echocardiography and myocardial deformation techniques in cardiac amyloidosis. Basis of the technique and diagnostically useful findings

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“…24 Apical sparing, a pattern characterized as LS reduction of the basal wall segment sparing apical segments, is calculated as the ratio between apical LS and basal and midventricle LS. 25,26 A ratio of 1 is thought to show high sensitivity (93%) and specificity (82%) for the diagnosis of cardiac amyloidosis 27 and independently predicts major adverse cardiac events. 28 Apical LS abnormalities are similar across different amyloidosis types and reflect the amyloid deposition burden.…”

Section: Echocardiogrammentioning

confidence: 99%

“…According to Pagourelias et al, the ejection fraction global longitudinal strain (LS) ratio has the best accuracy to detect cardiac amyloidosis in patients with ventricular hypertrophy; it has an estimated sensitivity of 89.7% and specificity of 91.7% 24 . Apical sparing, a pattern characterized as LS reduction of the basal wall segment sparing apical segments, is calculated as the ratio between apical LS and basal and mid‐ventricle LS 25,26 . A ratio of 1 is thought to show high sensitivity (93%) and specificity (82%) for the diagnosis of cardiac amyloidosis 27 and independently predicts major adverse cardiac events 28 .…”

Section: Diagnostic Approachmentioning

confidence: 99%

Diagnosis and treatment of transthyretin‐related amyloidosis cardiomyopathy

Teng

Bae

et al. 2020

Clinical Cardiology

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Transthyretin-related amyloidosis (ATTR) is a subgroup of amyloidosis that results from extracellular misassembled and toxic amyloid deposits affecting multiple organ systems, and cardiac tissues in particular. Because ATTR often presents as heart failure with preserved ejection fraction (HFpEF), it has been largely underdiagnosed. Once considered incurable with a grave prognosis, ATTR cardiomyopathy has seen the development of promising alternatives for diagnosis and treatment, with early diagnosis and treatment of ATTR cardiomyopathy highly beneficial due to its high mortality rate. For instance, diagnosing ATTR cardiomyopathy previously required a cardiac biopsy, but new modalities, such as cardiac magnetic resonance imaging and radionuclide bone scans, show promise in accurately diagnosing ATTR cardiomyopathy. Ongoing research and clinical trials have focused on identifying new treatments which primarily target amyloid fiber formation by inhibiting TTR gene expression, stabilizing the TTR tetramer, preventing oligomer aggregation, or affecting degradation of amyloid fibers. In this review, we describe the advances made in the diagnosis and treatment of ATTR in order to increase awareness of the disease and encourage a lower threshold for ATTR workup. Our review also highlights the need for improving the screening, diagnosis, and treatment guidelines for ATTR cardiomyopathy. K E Y W O R D S heart disease, physical diagnosis/cardiovascular, treatment 1 | BACKGROUND Transthyretin (TTR)-related amyloidosis (ATTR), a rare and underdiagnosed disease, mainly affects the cardiac and peripheral nerves and is fatal if not treated in time. 1 Primarily synthesized in the liver, TTR is a protein that transports vitamin A and thyroxine in plasma and cerebrospinal fluid. 2 The mechanism of transportation involves four oligomers that attach to each other to form two dimers, which in turn form a TTR tetramer that contains the hormone binding site. 2 Through gene mutation or aging of the protein, TTR can become unstable and break up into oligomers, which aggregate into insoluble fibrils called TTR amyloid that deposit into extracellular space or tissues, causing ATTR. 3 Based on the type of precursor and misfolded protein amyloids, there are different types of amyloid diseases from ATTR to light chain amyloidosis (AL), which is caused by aggregation of immunoglobulin light chains produced by plasma cells in the bone marrow. 4 Depending on the type of precursor protein, different protein amyloids target Catherine Teng and Pengyang Li contributed equally to this review article.

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The Imaging Diagnosis of Less Advanced Cases of Cardiac Amyloidosis: The Relative Apical Sparing Pattern

Cited by 7 publications

References 19 publications

Cardiac Amyloidosis

Cardiac Amyloidosis

Aportes del ecocardiograma y las técnicas de deformación miocárdica en la amiloidosis cardíaca. Bases de la técnica y hallazgos de utilidad diagnóstica

Diagnosis and treatment of transthyretin‐related amyloidosis cardiomyopathy

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