Cardiomyopathy: Clinical Diagnosis and Treatment

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

Deadline for manuscript submissions: closed (20 July 2023) | Viewed by 7943

Special Issue Editor


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Guest Editor
Department of Pediatrics, University of Toyama, Toyama, Japan
Interests: cardiomyopathy; left ventricualr noncomapction; genetics
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Special Issue Information

Dear Colleagues, 

Takotsubo syndrome, proposed in 1990, is a myocardial disorder characterized by asystole of the apex of the heart that develops in response to severe physical or mental stress. It is believed that severe physical or mental stress leads to a rapid increase in catecholamines in the body, resulting in coronary artery spasm and microvascular dysfunction, which in turn affects cardiomyocytes. Initially, it was thought to have a similar pathogenesis to acute coronary syndromes in terms of symptoms, electrocardiographic changes, and left ventricular wall motion abnormalities, but with fewer complications and a better prognosis than acute coronary syndromes because there is no coronary artery occlusion, and the wall motion abnormalities are reversible. However, as the concept of the disease has been widely recognized around the world and many research results have been reported, it has become clear that it presents with various cardiac complications. In recent years, it has also become clear that the prognosis is not always favorable. Thus, since the concept of Takotsubo syndrome was first introduced, a great deal of knowledge has been accumulated, and its clinical landscape is gradually becoming clearer, but the pathogenesis remains unclear. Moreover, the pathogenesis of the disease remains unclear, and its elucidation is awaited in order to establish treatment guidelines for this disease. In this Special Issue, we would like to present the latest findings on Takotsubo cardiomyopathy from various viewpoints and share this information with readers.

Dr. Keiichi Hirono
Guest Editor

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Keywords

  • Takatsubo syndrome
  • cardiomyopathy
  • genetics
  • heart failure
  • arrhythmia
  • sympathetic nervous system
  • catecholamine

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Published Papers (6 papers)

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Editorial

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3 pages, 165 KiB  
Editorial
Cardiomyopathy: A New Perspective from Diagnostic Strategy
by Keiichi Hirono
J. Clin. Med. 2023, 12(6), 2360; https://0-doi-org.brum.beds.ac.uk/10.3390/jcm12062360 - 18 Mar 2023
Viewed by 685
Abstract
Cardiomyopathy entails a broad group of diseases, acquired or genetic, which result in a similar phenotype [...] Full article
(This article belongs to the Special Issue Cardiomyopathy: Clinical Diagnosis and Treatment)

Research

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10 pages, 980 KiB  
Article
Predictive Factors for Decreasing Left Ventricular Ejection Fraction and Progression to the Dilated Phase of Hypertrophic Cardiomyopathy
by Kakeru Ishihara, Yoshiaki Kubota, Junya Matsuda, Yoichi Imori, Yukichi Tokita, Kuniya Asai and Hitoshi Takano
J. Clin. Med. 2023, 12(15), 5137; https://0-doi-org.brum.beds.ac.uk/10.3390/jcm12155137 - 05 Aug 2023
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Abstract
Patients with hypertrophic cardiomyopathy (HCM) may progress to the dilated phase (DHCM). This study aimed to identify the predictive factors for DHCM progression, including left ventricular (LV) ejection fraction (LVEF < 50%) or decreased LV contraction (LVEF < 60%). The study included 291 [...] Read more.
Patients with hypertrophic cardiomyopathy (HCM) may progress to the dilated phase (DHCM). This study aimed to identify the predictive factors for DHCM progression, including left ventricular (LV) ejection fraction (LVEF < 50%) or decreased LV contraction (LVEF < 60%). The study included 291 patients enrolled in our hospital’s HCM registry who were grouped based on their poststudy LVEF (LVEF of ≥60%, 50–59%, and <50%). Predictive factors of an LVEF of <50% or <60% were determined. Further, the effects of percutaneous transluminal septal myocardial ablation (PTSMA) on long-term systolic LV function and DHCM development were investigated. LVEF was ≥60%, 50–59%, and <50% in 239, 33, and 19 patients, respectively, during the follow-up period (mean: 64.9 months). Multivariate analyses indicated baseline atrial fibrillation (AF), nonsustained ventricular tachycardia (NSVT), and left ventricular diameter at end-systole (LVDs) as significant predictors of DHCM. Using a scoring method based on AF, NSVT, and LVDs, patients with 2 and 3 points had a significantly higher risk of developing DHCM. PTSMA in 78 HCM patients demonstrated no significant effect on long-term LVEF changes or DHCM development. We concluded that AF, NSVT, and LVDs are significant predictors of DHCM development. However, a validation study with a larger population is required. Full article
(This article belongs to the Special Issue Cardiomyopathy: Clinical Diagnosis and Treatment)
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9 pages, 609 KiB  
Article
Prognostic Impact of the Increase in Cardiac Troponin Levels during Tafamidis Therapy in Patients with Transthyretin Cardiac Amyloidosis
by Makiko Nakamura, Teruhiko Imamura, Ryuichi Ushijima and Koichiro Kinugawa
J. Clin. Med. 2023, 12(14), 4631; https://0-doi-org.brum.beds.ac.uk/10.3390/jcm12144631 - 12 Jul 2023
Cited by 2 | Viewed by 774
Abstract
Background: Recent clinical trials have demonstrated that tafamidis (Pfizer Inc., New York, NY, USA) reduced all-cause mortality and the number of cardiovascular hospitalizations compared with placebo in patients with transthyretin cardiac amyloidosis. However, the optimal surrogate markers during tafamidis treatment remain unknown. Methods: [...] Read more.
Background: Recent clinical trials have demonstrated that tafamidis (Pfizer Inc., New York, NY, USA) reduced all-cause mortality and the number of cardiovascular hospitalizations compared with placebo in patients with transthyretin cardiac amyloidosis. However, the optimal surrogate markers during tafamidis treatment remain unknown. Methods: Consecutive patients with transthyretin cardiac amyloidosis who received tafamidis in our institute between May 2019 and December 2022 were retrospectively evaluated. The prognostic impact of an increase in troponin I levels during tafamidis therapy was evaluated. Results: A total of 18 patients (median age 77 years, 84% male) were included. For 14-month tafamidis therapy on median, cardiac troponin I levels increased in five patients. The cumulative incidence of all-cause hospitalization was significantly higher in the troponin-increased group than in the others (100% versus 33%, p < 0.0001). Troponin increase was independently associated with the cumulative incidence of all-cause hospitalization with an adjusted hazard ratio of 5.14 (95% confidence interval 1.02–25.9, p = 0.048). Conclusions: The increase in cardiac troponin levels may be a reasonable surrogate marker of response to tafamidis therapy in patients with transthyretin cardiac amyloidosis. Full article
(This article belongs to the Special Issue Cardiomyopathy: Clinical Diagnosis and Treatment)
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12 pages, 826 KiB  
Article
Improvement of Left Ventricular Global Longitudinal Strain after 6-Month Therapy with GLP-1RAs Semaglutide and Dulaglutide in Type 2 Diabetes Mellitus: A Pilot Study
by Paolo Basile, Andrea Igoren Guaricci, Giuseppina Piazzolla, Sara Volpe, Alfredo Vozza, Marina Benedetto, Maria Cristina Carella, Daniela Santoro, Francesco Monitillo, Andrea Baggiano, Saima Mushtaq, Laura Fusini, Fabio Fazzari, Cinzia Forleo, Nunziata Ribecco, Gianluca Pontone, Carlo Sabbà and Marco Matteo Ciccone
J. Clin. Med. 2023, 12(4), 1586; https://0-doi-org.brum.beds.ac.uk/10.3390/jcm12041586 - 16 Feb 2023
Cited by 3 | Viewed by 2169
Abstract
(1) Background: Glucagone-Like Peptide-1 Receptor Agonists (GLP-1 RAs) (GLP-1 RAs) are incretine-based medications recommended in the treatment of type 2 Diabetes Mellitus (DM2) with atherosclerotic cardiovascular disease (ASCVD) or high or very high cardiovascular (CV) risk. However, knowledge of the direct mechanism of [...] Read more.
(1) Background: Glucagone-Like Peptide-1 Receptor Agonists (GLP-1 RAs) (GLP-1 RAs) are incretine-based medications recommended in the treatment of type 2 Diabetes Mellitus (DM2) with atherosclerotic cardiovascular disease (ASCVD) or high or very high cardiovascular (CV) risk. However, knowledge of the direct mechanism of GLP-1 RAs on cardiac function is modest and not yet fully elucidated. Left ventricular (LV) Global Longitudinal Strain (GLS) with Speckle Tracking Echocardiography (STE) represents an innovative technique for the evaluation of myocardial contractility. (2) Methods: an observational, perspective, monocentric study was conducted in a cohort of 22 consecutive patients with DM2 and ASCVD or high/very high CV risk, enrolled between December 2019 and March 2020 and treated with GLP-1 RAs dulaglutide or semaglutide. The echocardiographic parameters of diastolic and systolic function were recorded at baseline and after six months of treatment. (3) Results: the mean age of the sample was 65 ± 10 years with a prevalence of the male sex (64%). A significant improvement in the LV GLS (mean difference: −1.4 ± 1.1%; p value < 0.001) was observed after six months of treatment with GLP-1 RAs dulaglutide or semaglutide. No relevant changes were seen in the other echocardiographic parameters. (4) Conclusions: six months of treatment with GLP-1 RAs dulaglutide or semaglutide leads to an improvement in the LV GLS in subjects with DM2 with and high/very high risk for ASCVD or with ASCVD. Further studies on larger populations and with a longer follow-up are warranted to confirm these preliminary results. Full article
(This article belongs to the Special Issue Cardiomyopathy: Clinical Diagnosis and Treatment)
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8 pages, 1062 KiB  
Article
Dynamic Trend of Myocardial Edema in Takotsubo Syndrome: A Serial Cardiac Magnetic Resonance Study
by Ken Kato, Michiko Daimon, Masanori Sano, Koki Matsuno, Yoshiaki Sakai, Iwao Ishibashi, Tadayuki Kadohira, Koji Matsumoto, Yoshitada Masuda, Takashi Uno, Jelena-Rima Ghadri, Christian Templin and Yoshio Kobayashi
J. Clin. Med. 2022, 11(4), 987; https://0-doi-org.brum.beds.ac.uk/10.3390/jcm11040987 - 14 Feb 2022
Cited by 4 | Viewed by 1494
Abstract
Background: The wall motion abnormalities of the left ventricle (LV) in takotsubo syndrome (TTS) are known to be transient and completely recover within a few weeks. However, there is little information about the relationship between functional recovery and tissue characteristics. The aim of [...] Read more.
Background: The wall motion abnormalities of the left ventricle (LV) in takotsubo syndrome (TTS) are known to be transient and completely recover within a few weeks. However, there is little information about the relationship between functional recovery and tissue characteristics. The aim of this study was to investigate the recovery process of TTS using cardiovascular magnetic resonance (CMR). Methods: Consecutive patients with TTS were prospectively enrolled. We performed serial CMR in the acute phase (<72 h after admission), the subacute phase (7–10 days after admission) and the chronic phase (3 months later). To assess the degree of myocardial edema quantitatively, we evaluated the signal intensity of myocardium on T2-weighted images and calculated the signal intensity ratio compared with the skeletal muscle. Results: Fifteen patients with TTS were enrolled. CMR demonstrated reduced LV ejection fraction in the acute phase, and it recovered almost completely by the subacute phase. On the other hand, severe myocardial edema was still observed in the subacute phase, associated with increased LV mass. The highest signal intensity ratio in the subacute phase was correlated with the maximum voltage of negative T wave on electrocardiogram (r = 0.57, p = 0.03). Conclusions: In patients with TTS, myocardial edema associated with increased LV mass still remained in the subacute phase despite functional recovery of the LV. Electrocardiogram may be useful to assess the degree of myocardial edema in the subacute phase. Our study suggests that myocardial ischemia might have a central role in developing TTS. Full article
(This article belongs to the Special Issue Cardiomyopathy: Clinical Diagnosis and Treatment)
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Other

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5 pages, 643 KiB  
Case Report
A Novel NKX2-5 Variant in a Child with Left Ventricular Noncompaction, Atrial Septal Defect, Atrioventricular Conduction Disorder, and Syncope
by Yuya Yamada, Kazushi Yasuda, Yukiko Hata, Naoki Nishida and Keiichi Hirono
J. Clin. Med. 2022, 11(11), 3171; https://0-doi-org.brum.beds.ac.uk/10.3390/jcm11113171 - 02 Jun 2022
Cited by 2 | Viewed by 1208
Abstract
The NKX2-5 gene encodes a transcription factor and is actively involved in heart formation and development. A pediatric case with its variant and left ventricular noncompaction (LVNC) has not been reported. A 12-year-old girl with a history of a surgery for atrial septal [...] Read more.
The NKX2-5 gene encodes a transcription factor and is actively involved in heart formation and development. A pediatric case with its variant and left ventricular noncompaction (LVNC) has not been reported. A 12-year-old girl with a history of a surgery for atrial septal detect was referred because of syncope during exercise. The electrocardiogram showed atrioventricular block, and the echocardiogram revealed prominent trabeculations in the left ventricular wall, suggesting LVNC. A novel heterozygous variant in the NKX2-5 gene (NM_004387.1: c.255_256delCT, p.Phe86fs) was identified. NKX2-5 variants should be considered in cases with LVNC, congenital heart disease, arrhythmia, and syncope to prevent sudden cardiac death. Full article
(This article belongs to the Special Issue Cardiomyopathy: Clinical Diagnosis and Treatment)
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