Search for Articles:
Title / Keyword
Author / Affiliation / Email
Journal
Article Type
 
 
Advanced Search
 
Section
Special Issue
Volume
Issue
Number
Page
 
5.4
3.9
Journals
JCM
Special Issues
New Insights in Cardiac Amyloidosis and Its Complications
share announcement

New Insights in Cardiac Amyloidosis and Its Complications

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

Deadline for manuscript submissions: closed (26 November 2023) | Viewed by 6621

Special Issue Editors

Prof. Dr. Peter Luedike

E-Mail Website
Guest Editor
Department of Cardiology and Vascular Medicine, West German Heart and Vascular Center, University Hospital Essen, Essen, Germany
Interests: heart failure; cardiomyopathies; cardiogenic shock; cardiac physiology and hemodynamics of cardiac disease; interventional cardiology
Dr. Maria Papathanasiou

E-Mail Website
Guest Editor
Department of Cardiology and Vascular Medicine, West German Heart and Vascular Center, University Hospital Essen, Essen, Germany
Interests: heart failure; cardiomyopathies; mechanical circulatory support; heart transplantation

Special Issue Information

Dear Colleagues,

Cardiac amyloidosis is no longer a rare and exotic disease. Even if our understanding of the disease remains limited, tremendous progress has recently been made in diagnosing and treating this fatal disease. In this dedicated Special Issue we aim to provide new insights into the epidemiology, acute and chronic manifestations, as well as new and emerging therapies of cardiac amyloidosis and its complications. We welcome authors to submit their valuable work and contribute to demystifying this challenging clinical problem.

Prof. Dr. Peter Luedike
Dr. Maria Papathanasiou
Guest Editors

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

  • amyloidosis
  • light chains
  • tafamidis
  • transthyretin
  • restrictive cardiomyopathy
  • peripheral polyneuropathy
  • heart failure

Published Papers (6 papers)

Download All Papers
Order results
Result details
Show export options expand_more Show export options expand_less
Select all
Export citation of selected articles as:

Research

Jump to: Review

14 pages, 1574 KiB  
Article
High-Sensitivity Cardiac Troponin T to Exclude Cardiac Involvement in TTR Variant Carriers and ATTRv Amyloidosis Patients
by Hendrea S. A. Tingen, Milou Berends, Alwin Tubben, Johan Bijzet, Ewout J. Houwerzijl, Friso L. H. Muntinghe, Bart-Jan Kroesen, Paul A. van der Zwaag, Peter van der Meer, Riemer H. J. A. Slart, Bouke P. C. Hazenberg and Hans L. A. Nienhuis
J. Clin. Med. 2024, 13(3), 810; https://doi.org/10.3390/jcm13030810 - 30 Jan 2024
Viewed by 962
Abstract
(1) Background: Individuals carrying a pathogenic transthyretin gene variant (TTRv) are at high risk for developing hereditary transthyretin (ATTRv) amyloidosis and are routinely screened for the development of cardiomyopathy (ATTRv-CM). This study aims to evaluate whether the cardiac biomarkers N-terminal pro [...] Read more.
(1) Background: Individuals carrying a pathogenic transthyretin gene variant (TTRv) are at high risk for developing hereditary transthyretin (ATTRv) amyloidosis and are routinely screened for the development of cardiomyopathy (ATTRv-CM). This study aims to evaluate whether the cardiac biomarkers N-terminal pro B-type natriuretic peptide (NT-proBNP) and high-sensitivity cardiac troponin T (hs-cTnT) can be used to rule out ATTRv-CM. (2) Methods: In this retrospective case-control study, data from 46 ATTRv-CM patients and 101 TTRv carriers and ATTRv amyloidosis patients without cardiomyopathy were included. Binary logistic regression models were used to assess the ability of NT-proBNP and hs-cTnT to predict the diagnosis of ATTRv-CM. An optimal cutoff for the relevant biomarker(s) was determined based on a sensitivity of ≥99% and the highest possible percentage of additional tests avoided (%ATA) in the index dataset. (3) Results: Hs-cTnT demonstrated the highest predictive capabilities for ATTRv-CM. The addition of NT-proBNP did not improve the predictive model. A hs-cTnT cutoff of <6 ng/L resulted in a 97% sensitivity and a negative predictive value of 95% with a %ATA of 30% in the validation dataset. (4) Conclusion: In conclusion, hs-cTnT is a useful biomarker for excluding cardiac involvement in TTRv carriers and ATTRv amyloidosis patients and it has the potential to prevent unnecessary diagnostic procedures. Full article
(This article belongs to the Special Issue New Insights in Cardiac Amyloidosis and Its Complications)
►▼ Show Figures

Figure 1

13 pages, 2107 KiB  
Article
Multiparametric Monitoring of Disease Progression in Contemporary Patients with Wild-Type Transthyretin Amyloid Cardiomyopathy Initiating Tafamidis Treatment
by Svenja Ney, Roman Johannes Gertz, Lenhard Pennig, Richard J. Nies, Udo Holtick, Linus A. Völker, Gilbert Wunderlich, Katharina Seuthe, Christopher Hohmann, Clemens Metze, Claas Philip Nähle, Jennifer von Stein, Monique Brüwer, Henrik ten Freyhaus and Roman Pfister
J. Clin. Med. 2024, 13(1), 284; https://doi.org/10.3390/jcm13010284 - 04 Jan 2024
Viewed by 881
Abstract
Background: Recently, a disease modifying therapy has become available for transthyretin amyloid cardiomyopathy (ATTR-CM). A validated monitoring concept of treatment is lacking, but a current expert consensus recommends three clinical domains (clinical, biomarker and ECG/imaging) assessed by several measurable features to define disease [...] Read more.
Background: Recently, a disease modifying therapy has become available for transthyretin amyloid cardiomyopathy (ATTR-CM). A validated monitoring concept of treatment is lacking, but a current expert consensus recommends three clinical domains (clinical, biomarker and ECG/imaging) assessed by several measurable features to define disease progression. Methods: We retrospectively analyzed data of wild-type ATTR-CM patients initiating tafamidis therapy assessed within our local routine protocol at baseline and 6-months follow-up with respect to the frequency of values beyond the proposed thresholds defining disease progression. Additionally, associations of cardiac magnetic resonance (CMR) tomography with clinical domains were examined within a subgroup. Results: Sixty-two ATTR-CM patients were included (88.7% male, mean age 79 years). In total, 16.1% of patients had progress in the clinical and functional domain, 33.9% in the biomarker domain and 43.5% in the imaging/electrocardiography (ECG) domain, with the latter driven by deterioration of the diastolic dysfunction grade and global longitudinal strain. In total, 35.5% of patients showed progress in none, 35.5% in one, 29.0% in two and no patient in three domains, the latter indicating overall disease progression. A subgroup analysis of twenty-two patients with available baseline and follow-up CMR data revealed an increase in CMR-based extracellular volume by more than 5% in 18.2% of patients, with no significant correlation with progress in one of the clinical domains. Conclusions: We provide first frequency estimates of the markers of disease progression according to a recent expert consensus statement, which might help refine the multiparametric monitoring concept in patients with ATTR-CM. Full article
(This article belongs to the Special Issue New Insights in Cardiac Amyloidosis and Its Complications)
►▼ Show Figures

Figure 1

9 pages, 884 KiB  
Article
Safety and Tolerability of SGLT2 Inhibitors in Cardiac Amyloidosis—A Clinical Feasibility Study
by Maximilian J. Steinhardt, Vladimir Cejka, Mengmeng Chen, Sabrina Bäuerlein, Julia Schäfer, Ali Adrah, Sandra M. Ihne-Schubert, Aikaterini Papagianni, K. Martin Kortüm, Caroline Morbach and Stefan Störk
J. Clin. Med. 2024, 13(1), 283; https://doi.org/10.3390/jcm13010283 - 04 Jan 2024
Cited by 1 | Viewed by 1453
Abstract
Sodium-glucose transport protein 2 inhibitors (SGLT2i) slow the progression of renal dysfunction and improve the prognosis of patients with heart failure. Amyloidosis constitutes an important subgroup for which evidence is lacking. Amyloidotic fibrils originating from misfolded transthyretin and light chains are the causal [...] Read more.
Sodium-glucose transport protein 2 inhibitors (SGLT2i) slow the progression of renal dysfunction and improve the prognosis of patients with heart failure. Amyloidosis constitutes an important subgroup for which evidence is lacking. Amyloidotic fibrils originating from misfolded transthyretin and light chains are the causal agents in ATTR and AL amyloidosis. In these most frequent subtypes, cardiac involvement is the most common organ manifestation. Because cardiac and renal function frequently deteriorate over time, even under best available treatment, SGLT2i emerge as a promising treatment option due to their reno- and cardioprotective properties. We retrospectively analyzed patients with cardiac amyloidosis, who received either dapagliflozin or empagliflozin. Out of 79 patients, 5.1% had urinary tract infections; 2 stopped SGLT2i therapy; and 2.5% died unrelated to the intake of SGLT2i. No genital mycotic infections were observed. As expected, a slight drop in the glomerular filtration rate was noted, while the NYHA functional status, cardiac and hepatic function, as well as the 6 min walk distance remained stable over time. These data provide a rationale for the use of SGLT2i in patients with amyloidosis and concomitant cardiac or renal dysfunction. Prospective randomized data are desired to confirm safety and to prove efficacy in this increasingly important group of patients. Full article
(This article belongs to the Special Issue New Insights in Cardiac Amyloidosis and Its Complications)
►▼ Show Figures

Figure 1

11 pages, 779 KiB  
Article
Frailty in Older Patients with Transthyretin Cardiac Amyloidosis
by Stéphanie Cazalbou, Louise Naccache, Sandrine Sourdet, Eve Cariou, Pauline Fournier, Fati Nourhashemi, Laurent Balardy, Olivier Toulza, Olivier Lairez, Zara Steinmeyer and on behalf of the Toulouse Amyloidosis Research Network
J. Clin. Med. 2023, 12(24), 7507; https://doi.org/10.3390/jcm12247507 - 05 Dec 2023
Viewed by 825
Abstract
Background—Transthyretin cardiac amyloidosis (ATTR-CA) prevalence increases with age. The interplay between frailty and heart failure has been increasingly recognized. The objective of this study is to compare clinical, biological, and transthoracic echocardiography (TTE) characteristics of older ATTR-CA patients according to the G8 frailty [...] Read more.
Background—Transthyretin cardiac amyloidosis (ATTR-CA) prevalence increases with age. The interplay between frailty and heart failure has been increasingly recognized. The objective of this study is to compare clinical, biological, and transthoracic echocardiography (TTE) characteristics of older ATTR-CA patients according to the G8 frailty screening tool. Methods—Patients over 75 years old with a confirmed diagnosis of ATTR-CA were included between January 2020 and April 2021. All patients underwent a routine blood test, TTE, and a functional assessment with a six-minute walking distance test (6MWD) or cardiopulmonary exercise testing (CPET), and the G8 score was calculated. Results—Fifty-two patients were included. Thirty-nine (75%) patients were frail and their mean NYHA stage was more severe (2.2 vs. 1.7; p = 0.004); 62% of them had a Gilmore stage of 2 or 3 (p = 0.05). Global left ventricular strain (GLS) was lower (−11.7% vs. −14.9%; p = 0.014) and the interventricular septum was thicker (18 ± 2 mm vs. 17 ± 2 mm; p = 0.033) in frail patients. There were no significant differences according to functional tests. Conclusion—The majority of older patients with ATTR-CA are frail according to the G8 score. They are more symptomatic and have an increased cardiac involvement and a poorer prognosis, requiring more personalized cardiac management. Full article
(This article belongs to the Special Issue New Insights in Cardiac Amyloidosis and Its Complications)
►▼ Show Figures

Figure 1

18 pages, 1917 KiB  
Article
Risk Stratification in Transthyretin Cardiac Amyloidosis: The Added Value of Lung Spirometry
by Rishika Banydeen, Reid Eggleston, Antoine Deney, Astrid Monfort, Jay H. Ryu, Giuseppe Vergaro, Vincenzo Castiglione, Olivier Lairez, Michele Emdin, Jocelyn Inamo, Misbah Baqir and Remi Neviere
J. Clin. Med. 2023, 12(11), 3684; https://doi.org/10.3390/jcm12113684 - 26 May 2023
Viewed by 1037
Abstract
Transthyretin cardiac amyloidosis (ATTR-CA) is an increasingly recognized disease that often results in heart failure and death. Traditionally, biological staging systems are used to stratify disease severity. Reduced aerobic capacity has recently been described as useful in identifying higher risk of cardiovascular events [...] Read more.
Transthyretin cardiac amyloidosis (ATTR-CA) is an increasingly recognized disease that often results in heart failure and death. Traditionally, biological staging systems are used to stratify disease severity. Reduced aerobic capacity has recently been described as useful in identifying higher risk of cardiovascular events and death. Assessment of lung volume via simple spirometry might also hold prognostic relevance. We aimed to assess the combined prognostic value of spirometry, cardiopulmonary exercise testing (CPET) and biomarker staging in ATTR-CA patients in a multi-parametric approach. We retrospectively reviewed patient records with pulmonary function and CPET testing. Patients were followed until study endpoint (MACE: composite of heart-failure-related hospitalization and all-cause death) or censure (1 April 2022). In total, 82 patients were enrolled. Median follow-up was 9 months with 31 (38%) MACE. Impaired peak VO2 and forced vital capacity (FVC) were independent predictors of MACE-free survival, with peak VO2 < 50% and FVC < 70% defining the highest risk group (HR 26, 95% CI: 5–142, mean survival: 15 months) compared to patients with the lowest risk (peak VO2 ≥ 50% and FVC ≥ 70%). Combined peak VO2, FVC and ATTR biomarker staging significantly improved MACE prediction by 35% compared to ATTR staging alone, with 67% patients reassigned a higher risk category (p < 0.01). In conclusion, combining functional and biological markers might synergistically improve risk stratification in ATTR-CA. Integrating simple, non-invasive and easily applicable CPET and spirometry in the routine management of ATTR-CA patients might prove useful for improved risk prediction, optimized monitoring and timely introduction of newer-generation therapies. Full article
(This article belongs to the Special Issue New Insights in Cardiac Amyloidosis and Its Complications)
►▼ Show Figures

Figure 1

Review

Jump to: Research

14 pages, 2161 KiB  
Review
Cardiac Amyloidosis and Valvular Heart Disease
by Franz Duca, Christina Kronberger, Robin Willixhofer, Philipp E. Bartko, Jutta Bergler-Klein and Christian Nitsche
J. Clin. Med. 2024, 13(1), 221; https://doi.org/10.3390/jcm13010221 - 30 Dec 2023
Viewed by 989
Abstract
Growing interest has accrued in the co-existence of cardiac amyloidosis and valvular heart disease. Amyloid infiltration from either transthyretin (ATTR) or of light chain (AL) origin may affect any structure of the heart, including the valves. The recent literature has mainly focused on [...] Read more.
Growing interest has accrued in the co-existence of cardiac amyloidosis and valvular heart disease. Amyloid infiltration from either transthyretin (ATTR) or of light chain (AL) origin may affect any structure of the heart, including the valves. The recent literature has mainly focused on aortic stenosis and cardiac amyloidosis, improving our understanding of the epidemiology, diagnosis, treatment and prognosis of this dual pathology. Despite being of high clinical relevance, data on mitral/tricuspid regurgitation and cardiac amyloidosis are rather scarce and mostly limited to case reports and small cases series. It is the aim of this review article to summarize the current evidence of concomitant valvular heart disease and cardiac amyloidosis by including studies on epidemiology, diagnostic approaches, screening possibilities, therapeutic management, and prognostic implications. Full article
(This article belongs to the Special Issue New Insights in Cardiac Amyloidosis and Its Complications)
►▼ Show Figures

Figure A1

Show export options expand_more Show export options expand_less
Select all
Export citation of selected articles as:
 
Displaying articles 1-6
Back to TopTop