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Takotsubo Syndrome, Short QT Syndrome and Brugada Syndrome

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A special issue of Journal of Cardiovascular Development and Disease (ISSN 2308-3425). This special issue belongs to the section "Acquired Cardiovascular Disease".

Deadline for manuscript submissions: closed (15 February 2022) | Viewed by 13732

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Special Issue Editor

Dr. Xiaobo Zhou

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Guest Editor

First Department of Medicine, Medical Faculty Mannheim, University of Heidelberg, DZHK (German Center for Cardiovascular Research), Partner Site, Heidelberg-Mannheim, 68167 Mannheim, Germany
Interests: cellular electrophysiology; channelopathy; cardiomyopathy; arrhythmias

Special Issue Information

Dear Colleagues,

The Journal of Cardiovascular Development and Disease (JCDD) is launching a Special Issue focusing on Takotsubo Syndrome (TTS), short QT syndrome (SQTS), and Brugada Syndrome (BrS). TTS is a stress-associated heart disease, which is similar to acute coronary syndromes (ACS) but has no coronary stenosis. Usually, a regional left ventricular wall motion abnormality, leading to an abnormal shape of the left ventricle with a narrow neck and apical ballooning during systole, can be detected in the hearts of TTS patients. TTS was initially considered a benign disease. Later, studies showed that the severity of TTS was underestimated. SQTS and BrS are an inherited channelopathy with the risk of life-threatening arrhythmias and sudden cardiac death (SCD). SQTS represents abbreviated corrected QT interval (QTc). The ECG of BrS-patients shows a concave (coved type, type 1) or a convex (saddle-back type, type 2) ST-segment elevation. Catecholamine excess is widely believed to be a critical etiological factor for TTS, while mutations or variants in some ion channel genes are linked to SQTS and BrS. However, the exact pathomechanisms have not been clarified, and optimal therapy for these diseases is still lacking. Therefore, the interest in these fields is increasing, and rapid progress is expected. This Special Issue will provide a platform for the presentation of recent advances in pathomechanisms or therapy of TTS, SQTS, and BrS.

Dr. Xiaobo Zhou
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 Cardiovascular Development and Disease is an international peer-reviewed open access monthly 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 2700 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

Takotsubo syndrome mechanism
Takotsubo syndrome therapy
Short QT syndrome mechanism
Short QT syndrome therapy
Brugada syndrome mechanism
Brugada syndrome therapy

Published Papers (6 papers)

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Research

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11 pages, 1359 KiB

Open AccessArticle

Kidney Failure among Patients with Takotsubo Syndrome or Myocardial Infarction: A Retrospective Analysis

by Verena Bill, Ibrahim El-Battrawy, Marvin Kummer, Andreas Mügge, Assem Aweimer, Michael Behnes and Ibrahim Akin

J. Cardiovasc. Dev. Dis. 2022, 9(6), 186; https://0-doi-org.brum.beds.ac.uk/10.3390/jcdd9060186 - 09 Jun 2022

Viewed by 1601

Abstract

Background: Takotsubo syndrome (TTS) is a syndrome with ambiguous pathophysiology. Impaired kidney function (KF) seems to impact the outcome of patients with TTS. We hypothesized that KF worsens the outcome among TTS patients and furthermore, TTS patients with concomitant KF experience more adverse events compared to myocardial infarction (MI) patients with concomitant KF. Methods and Results: This retrospective single-center study comprised two groups (cohorts) of patients including patients with TTS and concomitant KF (n = 61, 27.1%) and patients with MI and concomitant KF (n = 164, 72.9%). The clinical outcomes were delineated as short-term outcomes defined as in-hospital adverse events during index hospitalization and long-term outcomes defined as adverse events over five-year clinical follow-ups. All-cause mortality, stroke, cardiopulmonary resuscitation (CPR), life-threatening arrhythmias, need for respiratory support, and cardiogenic shock with subsequent use of inotropic agents during index hospitalization were denoted as in-hospital adverse events. All-cause mortality, rehospitalization due to heart failure, stroke, thromboembolic events, and the recurrence of primary pathology (TTS and MI) were analyzed during five-year follow-ups after index hospitalization. A higher mortality rate was noted among TTS patients with KF compared to TTS without KF. In addition, in-hospital event rates in patients with TTS and concomitant KF compared to MI and concomitant KF were comparable with the exception of a higher rate of respiratory support in TTS patients. The mortality rate was significantly higher among patients with TTS and KF at 4 years (29.5% vs. 15.9%, p = 0.02) and 5 years (34.4% vs. 20.7%, p = 0.03) in comparison to patients with MI and concomitant KF. In contrast, the rate of re-hospitalization related to heart failure was higher at 30 days, and at one-, four-, and five-year follow-ups in patients suffering from MI and KF compared to TTS and concomitant KF. Additionally, the recurrence of MI after 4 and 5 years was higher than the recurrence of TTS (4.9% vs. 15.2%; 4.9% vs. 16.5%). There were no differences in life-threatening arrhythmias and stroke in both groups. Conclusions: Patients with TTS and concomitant KF have higher all-cause mortality when compared to MI and concomitant KF. The mechanisms responsible remain to be determined. Full article

(This article belongs to the Special Issue Takotsubo Syndrome, Short QT Syndrome and Brugada Syndrome)

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19 pages, 4183 KiB

Open AccessArticle

Lipopolysaccharide Modifies Sodium Current Kinetics through ROS and PKC Signalling in Induced Pluripotent Stem-Derived Cardiomyocytes from Brugada Syndrome Patient

by Zhenxing Liao, Yingrui Li, Xuehui Fan, Zhen Yang, Ibrahim El-Battrawy, Xiaobo Zhou and Ibrahim Akin

J. Cardiovasc. Dev. Dis. 2022, 9(4), 119; https://0-doi-org.brum.beds.ac.uk/10.3390/jcdd9040119 - 15 Apr 2022

Cited by 2 | Viewed by 2044

Abstract

Studies have suggested a connection between inflammation and arrhythmogenesis of Brugada syndrome (BrS). However, experimental studies regarding the roles of inflammation in the arrhythmogenesis of BrS and its underlying mechanism are still lacking. This study aimed to investigate the influence of inflammation on BrS-phenotype features using human-induced stem cell-derived cardiomyocytes (hiPSC-CMs) from a BrS-patient carrying an SCN10A variant (c.3749G > A). After LPS treatment, the peak sodium current decreased significantly in SCN10A-hiPSC-CMs, but not in healthy donor-hiPSC-CMs. LPS also changed sodium channel gating kinetics, including activation, inactivation, and recovery from inactivation. NAC (N-acetyl-l-cysteine), a blocker of ROS (reactive oxygen species), failed to affect the sodium current, but prevented the LPS-induced reduction of sodium channel currents and changes in gating kinetics, suggesting a contribution of ROS to the LPS effects. Hydrogen peroxide (H₂O₂), a main form of ROS in cells, mimicked the LPS effects on sodium channel currents and gating kinetics, implying that ROS might mediate LPS-effects on sodium channels. The effects of H₂O₂ could be attenuated by a PKC blocker chelerythrine, indicating that PKC is a downstream factor of ROS. This study demonstrated that LPS can exacerbate the loss-of-function of sodium channels in BrS cells. Inflammation may play an important role in the pathogenesis of BrS. Full article

(This article belongs to the Special Issue Takotsubo Syndrome, Short QT Syndrome and Brugada Syndrome)

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14 pages, 2786 KiB

Open AccessArticle

Antiarrhythmic Effects of Vernakalant in Human-Induced Pluripotent Stem Cell-Derived Cardiomyocytes from a Patient with Short QT Syndrome Type 1

by Qiang Xu, Xuemei Huang, Zenghui Meng, Yingrui Li, Rujia Zhong, Xin Li, Lukas Cyganek, Ibrahim El-Battrawy, Ibrahim Akin, Xiaobo Zhou and Huan Lan

J. Cardiovasc. Dev. Dis. 2022, 9(4), 112; https://0-doi-org.brum.beds.ac.uk/10.3390/jcdd9040112 - 09 Apr 2022

Cited by 1 | Viewed by 2077

Abstract

(1) Background: Short QT syndrome (SQTS) may result in sudden cardiac death. So far, no drugs, except quinidine, have been demonstrated to be effective in some patients with SQTS type 1 (SQTS1). This study was designed to examine the potential effectiveness of vernakalant for treating SQTS1 patients, using human-induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs) from a patient with SQTS1. (2) Methods: Patch clamp and calcium imaging techniques were used to examine the drug effects. (3) Results: Vernakalant prolonged the action potential duration (APD) in hiPSC-CMs from a SQTS1-patient (SQTS1-hiPSC-CMs). In spontaneously beating SQTS1-hiPSC-CMs, vernakalant reduced the arrhythmia-like events induced by carbachol plus epinephrine. Vernakalant failed to suppress the hERG channel currents but reduced the outward small-conductance calcium-activated potassium channel current. In addition, it enhanced Na/Ca exchanger currents and late sodium currents, in agreement with its APD-prolonging effect. (4) Conclusions: The results demonstrated that vernakalant can prolong APD and reduce arrhythmia-like events in SQTS1-hiPSC-CMs and may be a candidate drug for treating arrhythmias in SQTS1-patients. Full article

(This article belongs to the Special Issue Takotsubo Syndrome, Short QT Syndrome and Brugada Syndrome)

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9 pages, 1336 KiB

Open AccessArticle

A Case Series of Concomitant Cardiac Electrical Disease among Takotsubo Syndrome Patients and Literature Review

by Ibrahim El-Battrawy, Julia W. Erath, Mate Vamos, Assem Aweimer, Andreas Mügge, Siegfried Lang, Uzair Ansari, Thorsten Gietzen and Ibrahim Akin

J. Cardiovasc. Dev. Dis. 2022, 9(3), 79; https://0-doi-org.brum.beds.ac.uk/10.3390/jcdd9030079 - 09 Mar 2022

Cited by 1 | Viewed by 2169

Abstract

The pathophysiology of Takotsubo Syndrome (TTS) is not completely understood and the trigger of sudden cardiac death (SCD) in TTS is not clear either. We therefore sought to find an association between TTS and primary electrical diseases. A total of 148 TTS patients were analyzed between 2003 and 2017 in a bi-centric manner. Additionally, a literature review was performed. The patients were included in an ongoing retrospective cohort database. The coexistence of TTS and primary electrical diseases was confirmed in five cases as the following: catecholaminergic polymorphic ventricular tachycardia (CPVT, 18-year-old female) (n = 1), LQTS 1 (72-year-old female and 65-year-old female) (n = 2), LQTS 2 (17-year-old female) (n = 1), and LQTS in the absence of mutations (22-year-old female). Four patients suffered from malignant tachyarrhythmia and recurrent syncope after TTS. Except for the CPVT patient and one LQTS 1 patient, all other cases underwent subcutaneous ICD implantation. An event recorder of the CPVT patient after starting beta-blocker did not detect arrhythmias. The diagnosis of primary electrical disease was in 80% of cases unmasked on a TTS event. This diagnosis triggered a family clinical and genetic screening confirming the diagnosis of primary electrical disease. A subsequent literature review identified five cases as the following: a congenital atrioventricular block (n = 1), a Jervell and Lange-Nielsen Syndrome (n = 1), and a family LQTS in the absence of a mutation (n = 2), LQTS 2 (n = 1). A primary electrical disease should be suspected in young and old TTS patients with a family history of sudden cardiac death. In suspected cases, e.g., ongoing QT interval prolongation, despite recovery of left ventricular ejection fraction a family screening is recommended. Full article

(This article belongs to the Special Issue Takotsubo Syndrome, Short QT Syndrome and Brugada Syndrome)

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16 pages, 3484 KiB

Open AccessArticle

Glucose Counteracts Isoprenaline Effects on Ion Channel Functions in Human-Induced Pluripotent Stem Cell-Derived Cardiomyocytes

by Lin Qiao, Xuehui Fan, Zhen Yang, Ibrahim El-Battrawy, Xiaobo Zhou and Ibrahim Akin

J. Cardiovasc. Dev. Dis. 2022, 9(3), 76; https://doi.org/10.3390/jcdd9030076 - 04 Mar 2022

Viewed by 2126

Abstract

Recent studies indicate that the disorder of glucose metabolism in myocardial tissue is involved in the development of Takotsubo syndrome (TTS). This study investigated the effects of a high level of glucose on the pathogenesis of TTS, focusing on the electrophysiological mechanisms. Human-induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs) were treated with toxic concentration of isoprenaline (Iso, 1 mM) and a high level of glucose (22 mM) to mimic the setting of TTS and diabetes mellitus (DM). Iso prolonged action potential duration (APD) through enhancing the late sodium channel current and suppressing the transient outward potassium current (I_to). However, a high level of glucose prevented the APD prolongation and the change in I_to. High-level glucose reduced the expression levels of PI3K/Akt, β1-adrenoceptors, Gs-protein, and PKA, suggesting their involvement in the protective effects of high-level glucose against toxic effects of catecholamine. High glucose level did not influence Iso-induced ROS-generation, suggesting that the protective effects of high-level glucose against Iso did not result from changes in ROS generation. High-level glucose may protect cardiomyocytes from the toxic effects of catecholamine excess through suppressing β1-adrenoceptor-Gs-PKA signaling. DM may reduce the risk for occurrence of arrhythmias due to QT prolongation in TTS patients. Full article

(This article belongs to the Special Issue Takotsubo Syndrome, Short QT Syndrome and Brugada Syndrome)

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Review

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13 pages, 1999 KiB

Open AccessEditor’s ChoiceReview

The Arrhythmogenic Face of COVID-19: Brugada ECG Pattern in SARS-CoV-2 Infection

by Paul Zimmermann, Felix Aberer, Martin Braun, Harald Sourij and Othmar Moser

J. Cardiovasc. Dev. Dis. 2022, 9(4), 96; https://0-doi-org.brum.beds.ac.uk/10.3390/jcdd9040096 - 25 Mar 2022

Cited by 6 | Viewed by 3138

Abstract

In 1992, Brugada syndrome (BS) was first described; an often unrecognized cardiac conduction disorder mainly associated with unexplained sudden cardiac arrest and consecutive syncope. Nevertheless, the pathomechanism of BS and sudden cardiac death remains mainly explained. Mutations in the cardiac sodium channels, which cause a reduction or functional loss of these channels, are associated with characteristic electrocardiographic (ECG) abnormalities and malignant arrhythmia. The majority of affected people are previously healthy and unaware of their genetic predisposition for BS and might experience ventricular tachyarrhythmias and cardiac arrest potentially triggered by several factors (e.g., alcohol, sodium channel blockers, psychotropic drugs, and fever). Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) was firstly identified in Wuhan in early December 2019 and rapidly spread worldwide as coronavirus disease (COVID-19). COVID-19 is typically characterized by a severe inflammatory response, activation of the immune system, and high febrile illness. Due to this condition, symptomatic COVID-19 infection or vaccination might serve as inciting factor for unmasking the Brugada pattern and represents a risk factor for developing proarrhythmic complications. The aim of this narrative review was to detail the association between virus-related issues such as fever, electrolyte disturbance, and inflammatory stress of COVID-19 infection with transient Brugada-like symptoms and ECG-pattern and its susceptibility to proarrhythmogenic episodes. Full article

(This article belongs to the Special Issue Takotsubo Syndrome, Short QT Syndrome and Brugada Syndrome)

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