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

April 2022 - 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
6 Citations
4,707 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,
  • Adrian Crisan and
  • Nicolae Dan Tesloianu

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...

  • Article
  • Open Access
2 Citations
3,059 Views
19 Pages

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....

  • Case Report
  • Open Access
4 Citations
3,131 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,
  • John T. Bryant,
  • Kristin Swenson and
  • Paul M. Ford

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...

  • Perspective
  • Open Access
4 Citations
3,739 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...

  • Article
  • Open Access
9 Citations
4,554 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,
  • Konstantin Klein,
  • Christopher Hohmann,
  • Samuel Lee,
  • Isabel Breidert,
  • Alexander Hof and
  • Kaveh Eghbalzadeh
  • + 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...

  • Systematic Review
  • Open Access
11 Citations
7,751 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,
  • Michael E. Mitchell and
  • Aoy Tomita-Mitchell

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...

  • Article
  • Open Access
12 Citations
4,309 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,
  • Joshua Murphy,
  • Sawsan Awad and
  • John W. Bokowski

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...

  • Article
  • Open Access
4 Citations
3,138 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,
  • Rujia Zhong,
  • Xin Li,
  • Lukas Cyganek,
  • Ibrahim El-Battrawy,
  • Ibrahim Akin and
  • Xiaobo Zhou
  • + 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...

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