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Journal of Cardiovascular Development and Disease, Volume 9, Issue 4

2022 April - 31 articles

Cover Story: Traditional definitions of Ebstein’s anomaly (EA) and left ventricular noncompaction (LVNC), two rare congenital heart defects (CHDs), confine disease to either the right or left heart, respectively. However, around 15–29% of patients with EA commonly manifest with LVNC. Joint EA and LVNC (EA/LVNC) presentation encompasses characteristics of both CHDs and poses a higher risk of poor clinical outcomes. We queried PubMed, Medline, and Web of Science for all peer-reviewed publications from inception to February 2022 that discuss EA/LVNC and found 58 unique articles written in English. Here, we summarize the clinical and genetic understanding of EA/LVNC to date. We additionally postulate involvement of shared developmental pathways that may lead to this combined disease. View this paper
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Articles (31)

  • Review
  • Open Access
8 Citations
6,270 Views
15 Pages

Implantable Cardioverter Defibrillator in Primary and Secondary Prevention of SCD—What We Still Don′t Know

  • Andreea Maria Ursaru,
  • Antoniu Octavian Petris,
  • Irina Iuliana Costache,
  • Ana Nicolae and ,
  • Adrian Crisan and
  • Nicolae Dan Tesloianu
  • + 1 author+ 0 author

Implantable cardioverter defibrillators (ICDs) are the cornerstone of primary and secondary prevention of sudden cardiac death (SCD) all around the globe. In almost 40 years of technological advances and multiple clinical trials, there has been a con...

(This article belongs to the Section Electrophysiology and Cardiovascular Physiology)
  • Article
  • Open Access
4 Citations
3,435 Views
19 Pages

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

  • Zhenxing Liao,
  • Yingrui Li,
  • Xuehui Fan,
  • Zhen Yang and ,
  • Ibrahim El-Battrawy,
  • Xiaobo Zhou and
  • Ibrahim Akin
  • + 2 authors+ 0 author

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 article belongs to the Special Issue Takotsubo Syndrome, Short QT Syndrome and Brugada Syndrome)
  • Case Report
  • Open Access
5 Citations
3,648 Views
7 Pages

A Case Report of Severe Factor XI Deficiency during Cardiac Surgery: Less Can Be More

  • Toshinobu Kazui,
  • Vance G. Nielsen,
  • Spencer D. Audie,
  • Rajagopalan M. Venkataramani and ,
  • John T. Bryant,
  • Kristin Swenson and
  • Paul M. Ford
  • + 2 authors+ 0 author

Severe congenital Factor XI (FXI) deficiency (<20% normal activity) can be associated with significant bleeding disorders, and there has been great concern for severe bleeding following cardiac surgery requiring cardiopulmonary bypass (CPB) in thi...

(This article belongs to the Section Cardiac Surgery)
  • Perspective
  • Open Access
6 Citations
4,969 Views
6 Pages

Hypoplastic left heart syndrome (HLHS) is a genetically complex disorder. Whole genome sequencing enables comprehensive scrutiny of single nucleotide variants and small insertions/deletions, within both coding and regulatory regions of the genome, re...

(This article belongs to the Special Issue Hypoplastic Left Heart Syndrome: Etiology, Pathogenesis, Management and The Future)
  • Article
  • Open Access
10 Citations
5,253 Views
14 Pages

Feasibility and Comparison of Resting Full-Cycle Ratio and Computed Tomography Fractional Flow Reserve in Patients with Severe Aortic Valve Stenosis

  • Hendrik Wienemann,
  • Marcel C. Langenbach,
  • Victor Mauri,
  • Maryam Banazadeh and ,
  • Konstantin Klein,
  • Christopher Hohmann,
  • Samuel Lee,
  • Isabel Breidert,
  • Alexander Hof and
  • Matti Adam
  • + 11 authors+ 6 authors

Background: Computed tomography derived Fractional Flow Reserve (CT-FFR) has been shown to decrease the referral rate for invasive coronary angiography (ICA). The purpose of the study was to evaluate the diagnostic performance of CT-FFR compared to h...

(This article belongs to the Special Issue Transcatheter Aortic Valve Implantation (TAVI))
  • Systematic Review
  • Open Access
13 Citations
8,981 Views
18 Pages

A Systematic Review of Ebstein’s Anomaly with Left Ventricular Noncompaction

  • Suma K. Thareja,
  • Michele A. Frommelt,
  • Joy Lincoln,
  • John W. Lough and ,
  • Michael E. Mitchell and
  • Aoy Tomita-Mitchell
  • + 1 author+ 0 author

Traditional definitions of Ebstein’s anomaly (EA) and left ventricular noncompaction (LVNC), two rare congenital heart defects (CHDs), confine disease to either the right or left heart, respectively. Around 15–29% of patients with EA, whi...

(This article belongs to the Special Issue Congenital Heart Defects: Diagnosis, Management, and Treatment)
  • Article
  • Open Access
17 Citations
6,196 Views
17 Pages

A Novel Patent Ductus Arteriosus Severity Score to Predict Clinical Outcomes in Premature Neonates

  • Krishna Kishore Umapathi,
  • Brieann Muller,
  • Cyndi Sosnowski,
  • Aravind Thavamani and ,
  • Joshua Murphy,
  • Sawsan Awad and
  • John W. Bokowski
  • + 2 authors+ 0 author

Background: Patent Ductus Arteriosus (PDA) in premature neonates has been associated with comorbidities including chronic lung disease (CLD), and death. However, the treatment of PDA remains controversial. There have been several echocardiographic va...

  • Review
  • Open Access
4 Citations
3,763 Views
7 Pages

Background: Hypoplastic left heart syndrome (HLHS) is a rare but deadly form of human congenital heart disease, most likely of diverse etiologies. Hemodynamic alterations such as those resulting from premature foramen ovale closure or aortic stenosis...

(This article belongs to the Special Issue Hypoplastic Left Heart Syndrome: Etiology, Pathogenesis, Management and The Future)
  • Article
  • Open Access
6 Citations
3,606 Views
14 Pages

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

  • Qiang Xu,
  • Xuemei Huang,
  • Zenghui Meng,
  • Yingrui Li and ,
  • Rujia Zhong,
  • Xin Li,
  • Lukas Cyganek,
  • Ibrahim El-Battrawy,
  • Ibrahim Akin and
  • Huan Lan
  • + 6 authors+ 1 author

(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 effect...

(This article belongs to the Special Issue Takotsubo Syndrome, Short QT Syndrome and Brugada Syndrome)
  • Article
  • Open Access
6 Citations
5,657 Views
25 Pages

Inhibition of the NOTCH1 Pathway in the Stressed Heart Limits Fibrosis and Promotes Recruitment of Non-Myocyte Cells into the Cardiomyocyte Fate

  • Mohamed Nemir,
  • Maryam Kay,
  • Damien Maison,
  • Corinne Berthonneche and ,
  • Alexandre Sarre,
  • Isabelle Plaisance and
  • Thierry Pedrazzini
  • + 2 authors+ 0 author

Cardiac pathologies lead to an acute or gradual loss of cardiomyocytes. Because of the limited regenerative capacity of the mammalian heart, cardiomyocytes are only replaced by fibrotic tissue. Excessive fibrosis contributes to the deterioration of c...

(This article belongs to the Special Issue Model Systems for Heart Regeneration)
  • Review
  • Open Access
24 Citations
9,163 Views
17 Pages

Hypertension and Arrhythmias: A Clinical Overview of the Pathophysiology-Driven Management of Cardiac Arrhythmias in Hypertensive Patients

  • Jacopo Marazzato,
  • Federico Blasi,
  • Michele Golino,
  • Paolo Verdecchia and ,
  • Fabio Angeli and
  • Roberto De Ponti
  • + 1 author+ 0 author

Because of demographic aging, the prevalence of arterial hypertension (HTN) and cardiac arrhythmias, namely atrial fibrillation (AF), is progressively increasing. Not only are these clinical entities strongly connected, but, acting with a synergistic...

(This article belongs to the Special Issue Recent Advances in the Treatment of Hypertension)
  • Case Report
  • Open Access
2 Citations
3,625 Views
5 Pages

Pediatric Left Posteroseptal Accessory Pathway Ablation from Giant Coronary Sinus with Persistent Left Superior Cava

  • José Cruzalegui,
  • Sergi Cesar,
  • Oscar Campuzano,
  • Victoria Fiol and ,
  • Josep Brugada and
  • Georgia Sarquella-Brugada
  • + 1 author+ 0 author

We report a pediatric patient with persistent left superior vena cava and a D-transposition of great arteries, which is an uncommon relation. It is crucial to know the anatomy of the persistent left superior vena cava and the dilated coronary sinus t...

(This article belongs to the Special Issue Catheter Ablation of Cardiac Arrhythmias: Practices and Outcomes)
  • Review
  • Open Access
20 Citations
8,417 Views
15 Pages

The Role of Cardiac Magnetic Resonance in Aortic Stenosis and Regurgitation

  • Marco Guglielmo,
  • Chiara Rovera,
  • Mark G. Rabbat and
  • Gianluca Pontone

Cardiac magnetic resonance (CMR) imaging is a well-set diagnostic technique for assessment of valvular heart diseases and is gaining ground in current clinical practice. It provides high-quality images without the administration of ionizing radiation...

(This article belongs to the Special Issue Cardiac Imaging in Valvular Heart Disease)
  • Perspective
  • Open Access
4 Citations
3,564 Views
6 Pages

The development and translation of regenerative medicine approaches for the treatment of hypoplastic left heart syndrome (HLHS) provides a promising alternative to the current standard of care. We review the strategies that have been pursued to date...

(This article belongs to the Special Issue Hypoplastic Left Heart Syndrome: Etiology, Pathogenesis, Management and The Future)
  • Review
  • Open Access
16 Citations
5,768 Views
16 Pages

Exaggerated cardiovascular reactivity to and delayed recovery from stress increase the risk of cardiovascular diseases in the future. While exercise training has been shown to attenuate stress-induced cardiovascular reactivity and enhance recovery fr...

(This article belongs to the Special Issue Cardiovascular Effects and Benefits of Exercise)
  • Article
  • Open Access
2 Citations
2,808 Views
8 Pages

Old age has been proven to be related to progressed arterial or aortic stiffness. Aortic stiffness is an independent predictor of all-cause and cardiovascular disease mortalities in patients who have undergone coronary artery bypass grafting (CABG) s...

  • Article
  • Open Access
6 Citations
3,932 Views
11 Pages

The renin-angiotensin system (RAS) is a crucial regulator of vascular resistance and blood volume in the body. This study aimed to examine the genetic predisposition of the plasma renin concentration influencing future hypertension incidence. Based o...

(This article belongs to the Section Genetics)
  • Systematic Review
  • Open Access
25 Citations
6,983 Views
13 Pages

Clinical Presentation and Management of Methicillin-Resistant Staphylococcus aureus Pericarditis—Systematic Review

  • Milan Radovanovic,
  • Marija Petrovic,
  • Richard D. Hanna,
  • Charles W. Nordstrom and ,
  • Andrew D. Calvin,
  • Michel K. Barsoum,
  • Natasa Milosavljevic,
  • Djordje Jevtic,
  • Mladen Sokanovic and
  • Igor Dumic
  • + 5 authors+ 0 author

In the expanding era of antibiotic resistance, new strains of Staphylococcus aureus have emerged which possess resistance to traditionally used antibiotics (MRSA). Our review aimed to systematically synthesize information on previously described MRSA...

  • Article
  • Open Access
2 Citations
3,105 Views
7 Pages

Vitamin D Related Gene Polymorphisms and Cholesterol Levels in a Mediterranean Population

  • Hana M. A. Fakhoury,
  • Said El Shamieh,
  • Amru Rifai,
  • Hani Tamim and
  • Rajaa Fakhoury

In addition to its role in bone health, vitamin D (VitD) has been implicated in several pathological conditions. Specifically, VitD deficiency has been linked to an increased risk of dyslipidemia. Atherogenic dyslipidemia is characterized by increase...

  • Article
  • Open Access
11 Citations
7,690 Views
12 Pages

Optimal Landmark for Chest Compressions during Cardiopulmonary Resuscitation Derived from a Chest Computed Tomography in Arms-Down Position

  • Pimpan Usawasuraiin,
  • Borwon Wittayachamnankul,
  • Boriboon Chenthanakij,
  • Juntima Euathrongchit and ,
  • Phichayut Phinyo and
  • Theerapon Tangsuwanaruk
  • + 1 author+ 0 author

Compressions at the left ventricle increase rate of return of spontaneous circulation. This study aimed to identify the landmark of the point of maximal left ventricular diameter on the sternum (LVmax) by using chest computed tomography (CCT) in the...

(This article belongs to the Special Issue Interventional Therapies and Management in Coronary Artery Disease)
  • Article
  • Open Access
3 Citations
7,107 Views
6 Pages

Acute Effect of Evolocumab on Lipoprotein(a) Level and Inflammation in Patients with Coronary Artery Disease

  • Seung Woo Choi,
  • Joan Kim,
  • Gyeong Won Jang,
  • Young Shin Lee and ,
  • Jin Sun Park,
  • Jung Myung Lee,
  • Hyung Oh Kim,
  • Hyemoon Chung,
  • Jong Shin Woo and
  • Weon Kim
  • + 6 authors+ 1 author

Background: Several studies have shown that high plasma lipoprotein(a) concentrations are associated with an increased risk of arteriosclerotic cardiovascular disease. Thus, Lp(a) has emerged as a new therapeutic target. Circulating proprotein conver...

  • Article
  • Open Access
18 Citations
8,991 Views
12 Pages

Efficacy and Safety of Acute Phase Intensive Electrical Muscle Stimulation in Frail Older Patients with Acute Heart Failure: Results from the ACTIVE-EMS Trial

  • Shinya Tanaka,
  • Kentaro Kamiya,
  • Yuya Matsue,
  • Ryusuke Yonezawa and ,
  • Hiroshi Saito,
  • Nobuaki Hamazaki,
  • Ryota Matsuzawa,
  • Kohei Nozaki,
  • Masashi Yamashita and
  • Junya Ako
  • + 11 authors+ 6 authors

As frailty in older patients with acute heart failure (AHF) has an adverse effect on clinical outcomes, the addition of electrical muscle stimulation (EMS) to exercise-based early rehabilitation may improve the effects of treatment. Post hoc analysis...

(This article belongs to the Special Issue Cardiac Rehabilitation: State of the Art and Perspectives)
  • Review
  • Open Access
20 Citations
5,145 Views
11 Pages

Arrhythmogenic cardiomyopathy (ACM) is a cardiomyopathy characterized by the occurrence of a high risk of life-threatening ventricular arrhythmias and sudden cardiac death even at presentation. Diagnosis, evolution and outcomes in adults have been ex...

(This article belongs to the Special Issue Pediatric Cardiomyopathies: From Genotype to Phenotype)
  • Article
  • Open Access
14 Citations
6,672 Views
11 Pages

Acute-Phase Initiation of Cardiac Rehabilitation for Short-Term Improvement in Activities of Daily Living in Patients Hospitalized for Acute Heart Failure

  • Kensuke Ueno,
  • Kentaro Kamiya,
  • Hidehiro Kaneko,
  • Akira Okada and ,
  • Hidetaka Itoh,
  • Katsuhito Fujiu,
  • Norifumi Takeda,
  • Hiroyuki Morita,
  • Nobuaki Michihata and
  • Issei Komuro
  • + 7 authors+ 2 authors

Background: Whether acute-phase cardiac rehabilitation (CR) is beneficial for short-term improvement in activities of daily living (ADL) in patients hospitalized for acute heart failure (AHF) remains unclear. Aim: To investigate the association of ac...

(This article belongs to the Special Issue Cardiac Rehabilitation: State of the Art and Perspectives)
  • Review
  • Open Access
9 Citations
6,051 Views
13 Pages

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

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

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 re...

(This article belongs to the Special Issue Takotsubo Syndrome, Short QT Syndrome and Brugada Syndrome)
  • Article
  • Open Access
14 Citations
5,229 Views
13 Pages

Background: At present, the effects of discharge modes of multielectrode catheters on the distribution of pulsed electric fields have not been completely clarified. Therefore, the control of the distribution of the pulsed electric field by selecting...

(This article belongs to the Special Issue Catheter Ablation of Cardiac Arrhythmias: Practices and Outcomes)
  • Review
  • Open Access
46 Citations
11,653 Views
10 Pages

Pulsed Field Ablation to Treat Atrial Fibrillation: A Review of the Literature

  • Antonio Di Monaco,
  • Nicola Vitulano,
  • Federica Troisi,
  • Federico Quadrini and ,
  • Imma Romanazzi,
  • Valeria Calvi and
  • Massimo Grimaldi
  • + 2 authors+ 0 author

Atrial fibrillation (AF) is the most common sustained cardiac arrhythmia and catheter ablation, which can be used in symptomatic patients refractory to antiarrhythmic therapy. Pulmonary vein isolation (PVI) remains the cornerstone of any ablation pro...

  • Review
  • Open Access
6 Citations
7,261 Views
11 Pages

Porcine Models of Heart Regeneration

  • Nivedhitha Velayutham and
  • Katherine E. Yutzey

Swine are popular large mammals for cardiac preclinical testing due to their similarities with humans in terms of organ size and physiology. Recent studies indicate an early neonatal regenerative capacity for swine hearts similar to small mammal labo...

(This article belongs to the Special Issue Model Systems for Heart Regeneration)
  • Editorial
  • Open Access
3,647 Views
9 Pages

This Introduction provides both a short reflection on the scientific career of Adriana Gittenberger-de Groot and an overview of the papers that form the basis of this Special Issue giving them a proper perspective. The papers have as a central focus...

(This article belongs to the Special Issue Leaders in Cardiovascular Research: A Special Issue Dedicated to Professor Adriana Gittenberger-De Groot)
  • Article
  • Open Access
2 Citations
4,496 Views
12 Pages

Slow-Pathway Visualization by Using Panoramic View: A Novel Ablation Technique for Ablation of Atrioventricular Nodal Reentrant Tachycardia

  • Lei Ding,
  • Sixian Weng,
  • Hongda Zhang,
  • Fengyuan Yu and ,
  • Yingjie Qi,
  • Shu Zhang and
  • Min Tang
  • + 2 authors+ 0 author

(1) Background: The panoramic view of a novel wide-band dielectric mapping system could show the individual anatomy. We aimed to compare the feasibility, efficacy and safety of the panoramic view guided approach for ablation of AVNRT with the convent...

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J. Cardiovasc. Dev. Dis. - ISSN 2308-3425