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Diagnosis and Management of Cardiac Amyloidosis
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Diagnosis and Management of Cardiac Amyloidosis

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Cardiology".

Deadline for manuscript submissions: closed (31 December 2021) | Viewed by 7850

Special Issue Editor

Prof. Dr. Olivier Lairez

E-Mail Website
Guest Editor
Department of Cardiology, University Hospital of Toulouse, Toulouse, France
Interests: cardiac amyloidosis; hypertrophic cardiomyopathy; heart failure; cardiac imaging

Special Issue Information

Dear Colleagues,

Cardiac amyloidosis has undergone major changes in recent years: first with the development of non-invasive diagnostic techniques that have led to an increase in diagnoses and, more recently, the development and improvement of treatments, particularly in the field of transthyretin amyloidosis. These developments have led the entire cardiology community to take an interest in this pathology and to set up collaborations between specialists to improve diagnosis and management. Cardiac amyloidosis leads us to reconsider cardiology from a new perspective, with a pathology that differs in all respects from other cardiomyopathies and requires dedicated knowledge and multidisciplinary management. For all these reasons, it seemed necessary to create a Special Issue on this topic. With this Special Issue, we hope to encourage submissions that discuss the current state of the art, address ongoing knowledge gaps, and focus on ongoing controversies and management related to cardiac amyloidosis.

Prof. Dr. Olivier Lairez
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Journal of Clinical Medicine is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • Cardiac amyloidosis
  • Transthyretin amyloidosis
  • Light chain amyloidosis
  • Heart failure
  • Diagnosis
  • Management

Published Papers (3 papers)

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Research

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11 pages, 1467 KiB  
Article
Echocardiographic Patterns of Left Ventricular Diastolic Function in Cardiac Amyloidosis: An Updated Evaluation
by Silvia Oghina, Wulfran Bougouin, Mounira Kharoubi, Louis Bonnefous, Arnault Galat, Soulef Guendouz, Mélanie Bezard, Fabien Le Bras, Jean-François Deux, Emmanuel Itti, Anissa Moktefi, Pascale Fanen, Emmanuel Teiger, Dania Mohty, Thibaud Damy and Diane Bodez
J. Clin. Med. 2021, 10(21), 4888; https://doi.org/10.3390/jcm10214888 - 23 Oct 2021
Cited by 4 | Viewed by 1994
Abstract
Aims: Multimodal imaging has allowed cardiac amyloidosis (CA) to be increasingly recognised as a treatable cause of heart failure with preserved ejection fraction, but its prognosis remains poor due to late diagnosis. To assess the left ventricular diastolic function (LVDF) patterns in a [...] Read more.
Aims: Multimodal imaging has allowed cardiac amyloidosis (CA) to be increasingly recognised as a treatable cause of heart failure with preserved ejection fraction, but its prognosis remains poor due to late diagnosis. To assess the left ventricular diastolic function (LVDF) patterns in a large contemporary CA cohort according to the current recommendations and to identify their determinants. Methods and Results: We conducted a monocentric, observational study on a cohort of CA patients from a tertiary CA referral centre. Diastolic function was analysed using standard echocardiography and clinical, laboratory and survival parameters were collected. Four hundred and sixty-four patients with one of the three main type of CA were included: 41% had grade III diastolic dysfunction (restrictive mitral pattern), 25% had grade II diastolic dysfunction, and 25% had grade I diastolic dysfunction; 9% were unclassified. No difference was found between the main CA types. After multivariate analyses, grades II and III were independently associated with dyspnoea, elevated NT-proBNP, cardiac infiltration and systolic dysfunction (global longitudinal strain). Grade I patients had a better prognosis. Conclusions: All LVDF patterns can be observed in CA. One quarter of CA patients have grade I LVDF, reflecting the emergence of earlier stage-related phenotypes with a better prognosis. Full article
(This article belongs to the Special Issue Diagnosis and Management of Cardiac Amyloidosis)
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15 pages, 3156 KiB  
Article
Prognostic Value of N-Terminal Pro-Brain Natriuretic Peptide and High-Sensitivity Troponin T Levels in the Natural History of Transthyretin Amyloid Cardiomyopathy and Their Evolution after Tafamidis Treatment
by Silvia Oghina, Constant Josse, Mélanie Bézard, Mounira Kharoubi, Marc-Antoine Delbarre, Damien Eyharts, Amira Zaroui, Soulef Guendouz, Arnault Galat, Luc Hittinger, Pascale Fanen, Emmanuel Teiger, Nadir Mouri, François Montestruc and Thibaud Damy
J. Clin. Med. 2021, 10(21), 4868; https://doi.org/10.3390/jcm10214868 - 22 Oct 2021
Cited by 9 | Viewed by 2428
Abstract
Background: We assesse the evolution and prognostic value of N-terminal pro-brain natriuretic peptide (NT-proBNP) and high-sensitivity troponin T (cTnT-HS) in transthyretin amyloid cardiomyopathy (ATTR-CA) before and after tafamidis treatment. Methods and Results: 454 ATTR-CA patients without tafamidis (Cohort A) and 248 ATTR-CA with [...] Read more.
Background: We assesse the evolution and prognostic value of N-terminal pro-brain natriuretic peptide (NT-proBNP) and high-sensitivity troponin T (cTnT-HS) in transthyretin amyloid cardiomyopathy (ATTR-CA) before and after tafamidis treatment. Methods and Results: 454 ATTR-CA patients without tafamidis (Cohort A) and 248 ATTR-CA with tafamidis (Cohort B) were enrolled. Event-free survival (EFS) events were death, heart transplant, or acute heart failure. In Cohort A, 27% of patients maintained NT-proBNP < 3000 ng/L and 14% cTnT-HS < 50 ng/L at 12 months relative to baseline levels. In Cohort B, the proportions were 49% and 29%, respectively. In Cohort A, among the 333 patients without an increased NT-proBNP > 50% relative to baseline EFS was extended compared to the 121 patients with an increased NT-proBNP > 50% (HR: 0.75 [0.57; 0.98]; p = 0.032). In Cohort A, baseline NT-proBNP > 3000 ng/L and cTnT-HS > 50 ng/L and a relative increase of NT-proBNP > 50% during follow-up were independent prognostic factors of EFS. The slopes of logs NT-proBNP and cTnT-HS increased with time before and stabilized after tafamidis. Conclusion: ATTR-CA patients with increasing NT-proBNP had an increased risk of EFS. Tafamidis stabilize NT-proBNP and cTnT-HS increasing, even if initial NT-proBNP levels were >3000 ng/L. Thus suggesting that all patients, irrespective of baseline NT-proBNP levels, may benefit from tafamidis. Full article
(This article belongs to the Special Issue Diagnosis and Management of Cardiac Amyloidosis)
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Review

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15 pages, 1055 KiB  
Review
Physiology, Diagnosis and Treatment of Cardiac Light Chain Amyloidosis
by Agnieszka Stelmach-Gołdyś, Monika Zaborek-Łyczba, Jakub Łyczba, Bartosz Garus, Marcin Pasiarski, Paulina Mertowska, Paulina Małkowska, Rafał Hrynkiewicz, Paulina Niedźwiedzka-Rystwej and Ewelina Grywalska
J. Clin. Med. 2022, 11(4), 911; https://doi.org/10.3390/jcm11040911 - 9 Feb 2022
Cited by 6 | Viewed by 2539
Abstract
AL (light-chain) amyloidosis is a systemic disease in which amyloid fibers are formed from kappa or lambda immunoglobulin light chains, or fragments thereof, produced by a neoplastic clone of plasmocytes. The produced protein is deposited in tissues and organs in the form of [...] Read more.
AL (light-chain) amyloidosis is a systemic disease in which amyloid fibers are formed from kappa or lambda immunoglobulin light chains, or fragments thereof, produced by a neoplastic clone of plasmocytes. The produced protein is deposited in tissues and organs in the form of extracellular deposits, which leads to impairment of their functions and, consequently, to death. Despite the development of research on pathogenesis and therapy, the mortality rate of patients with late diagnosed amyloidosis is 30%. The diagnosis is delayed due to the complex clinical picture and the slow progression of the disease. This is the type of amyloidosis that most often contributes to cardiac lesions and causes cardiac amyloidosis (CA). Early diagnosis and correct identification of the type of amyloid plays a crucial role in the planning and effectiveness of therapy. In addition to standard histological studies based on Congo red staining, diagnostics are enriched by tests to determine the degree of cardiac involvement. In this paper, we discuss current diagnostic methods used in cardiac light chain amyloidosis and the latest therapies that contribute to an improved patient prognosis. Full article
(This article belongs to the Special Issue Diagnosis and Management of Cardiac Amyloidosis)
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