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14 Results Found

  • Case Report
  • Open Access
1,597 Views
16 Pages

Severe Aortic Stenosis and Pre-Excitation Syndrome in Pregnancy—A Multidisciplinary Approach

  • Miruna Florina Ştefan,
  • Lucia Ştefania Magda,
  • Catalin Gabriel Herghelegiu,
  • Doru Herghelegiu,
  • Oana Aurelia Zimnicaru,
  • Catalin Constantin Badiu,
  • Maria Claudia Berenice Suran,
  • Andreea Elena Velcea,
  • Calin Siliste and
  • Dragoș Vinereanu

20 August 2025

Background/Objectives: Heart disease affects 0.1% to 4% of pregnant women, with congenital heart defects being the leading cause in developed countries. While maternal mortality is generally low, pre-existing cardiac conditions substantially increase...

  • Article
  • Open Access
1 Citations
1,278 Views
13 Pages

Spontaneous Resolution of Ventricular Pre-Excitation During Childhood: A Retrospective Study

  • Antonio Sanzo,
  • Alessandro Seganti,
  • Andrea Demarchi,
  • Riccardo Simone Fino,
  • Irene Raso,
  • Alessia Claudia Codazzi,
  • Barbara Petracci,
  • Andrea Bongiorno,
  • Roberto Rordorf and
  • Savina Mannarino

29 March 2025

Background/Objectives: Ventricular pre-excitation (VP) increases the risk of sudden cardiac death among children. While transcatheter ablation could potentially be therapeutic, it is not without risk, especially in smaller children. Accessory pathway...

  • Review
  • Open Access
5 Citations
3,906 Views
10 Pages

Intrafamilial Phenotypical Variability Linked to PRKAG2 Mutation—Family Case Report and Review of the Literature

  • Andreea Sorina Marcu,
  • Radu Vătăşescu,
  • Sebastian Onciul,
  • Viorica Rădoi and
  • Ruxandra Jurcuţ

18 December 2022

PRKAG2 syndrome (PS) is a rare, early-onset autosomal dominant phenocopy of sarcomeric hypertrophic cardiomyopathy (HCM), that mainly presents with ventricular pre-excitation, cardiac hypertrophy and progressive conduction system degeneration. Its na...

  • Article
  • Open Access
11 Citations
3,727 Views
8 Pages

Identifying the Location of an Accessory Pathway in Pre-Excitation Syndromes Using an Artificial Intelligence-Based Algorithm

  • Thomas Senoner,
  • Bernhard Pfeifer,
  • Fabian Barbieri,
  • Agne Adukauskaite,
  • Wolfgang Dichtl,
  • Axel Bauer and
  • Florian Hintringer

26 September 2021

(1) Background: The exact anatomic localization of the accessory pathway (AP) in patients with Wolff–Parkinson–White (WPW) syndrome still relies on an invasive electrophysiologic study, which has its own inherent risks. Determining the AP localizatio...

  • Interesting Images
  • Open Access
249 Views
3 Pages

We present the case of a young male presenting with a wide complex tachycardia. The diagnostic approach based on the electrocardiogram findings is discussed. The final diagnosis could be made following an adenosine challenge at baseline, underlying t...

  • Review
  • Open Access
16 Citations
21,127 Views
18 Pages

Wolf–Parkinson–White Syndrome: Diagnosis, Risk Assessment, and Therapy—An Update

  • Radu Gabriel Vătășescu,
  • Cosmina Steliana Paja,
  • Ioana Șuș,
  • Simona Cainap,
  • Ștefana María Moisa and
  • Eliza Elena Cinteză

Wolf–Parkinson–White (WPW) syndrome is a disorder characterized by the presence of at least one accessory pathway (AP) that can predispose people to atrial/ventricular tachyarrhythmias and even sudden cardiac death. It is the second most...

  • Feature Paper
  • Article
  • Open Access
9 Citations
5,050 Views
16 Pages

Danon Disease: Entire LAMP2 Gene Deletion with Unusual Clinical Presentation—Case Report and Review of the Literature

  • Adel Shalata,
  • Marina Bar-Shai,
  • Yarin Hadid,
  • Muhammad Mahroum,
  • Hila Mintz,
  • Zaher Eldin Shalata,
  • Evgeny Radzishevsky,
  • Jacob Genizi,
  • Avraham Lorber and
  • Liat Yaniv
  • + 1 author

27 July 2023

Danon disease is a rare x-linked dominant multisystemic disorder with a clinical triad of severe cardiomyopathy, skeletal myopathy, and intellectual disability. It is caused by defects in the lysosome-associated membrane protein-2 (LAMP2) gene. Numer...

  • Case Report
  • Open Access
2,116 Views
22 Pages

When Paying Attention Pays Back: Missense Mutation c.1006G>A p. (Val336Ile) in PRKAG2 Gene Causing Left Ventricular Hypertrophy and Conduction Abnormalities in a Caucasian Patient: Case Report and Literature Review

  • Emanuele Micaglio,
  • Lara Tondi,
  • Sara Benedetti,
  • Maria Alessandra Schiavo,
  • Antonia Camporeale,
  • Giandomenico Disabato,
  • Andrea Attanasio,
  • Gianluigi Guida,
  • Gianpaolo Carrafiello and
  • Massimo Lombardi
  • + 3 authors

23 August 2024

PRKAG2 cardiomyopathy is a rare genetic disorder that manifests early in life with an autosomal dominant inheritance pattern. It harbors left ventricular hypertrophy (LVH), ventricular pre-excitation and progressively worsening conduction system defe...

  • Article
  • Open Access
1 Citations
2,327 Views
13 Pages

PRKAG2 Syndrome: Clinical Features, Imaging Findings and Cardiac Events

  • Maria Sudomir,
  • Przemysław Chmielewski,
  • Grażyna Truszkowska,
  • Mariusz Kłopotowski,
  • Mateusz Śpiewak,
  • Marta Legatowicz-Koprowska,
  • Monika Gawor-Prokopczyk,
  • Justyna Szczygieł,
  • Joanna Zakrzewska-Koperska and
  • Zofia T. Bilińska
  • + 4 authors

Background/Objectives: PRKAG2 syndrome (PS) is a rare genocopy of hypertrophic cardiomyopathy (HCM). Our goal was to expand knowledge about PS by analyzing patient clinical, imaging, and follow-up data. Methods: The study included carriers of li...

  • Article
  • Open Access
3 Citations
2,778 Views
8 Pages

Accuracy of Algorithms Predicting Accessory Pathway Localization in Pediatric Patients with Wolff-Parkinson-White Syndrome

  • Stefan Kurath-Koller,
  • Martin Manninger,
  • Nathalie Öffl,
  • Martin Köstenberger,
  • Hannes Sallmon,
  • Joachim Will and
  • Daniel Scherr

14 December 2022

We aimed to assess the accuracy of determining accessory pathway (AP) localization from 12 lead ECG tracings by applying 12 different algorithms in pediatric patients diagnosed with Wolff-Parkinson-White syndrome. We compared algorithm accuracy in el...

  • Review
  • Open Access
7 Citations
8,658 Views
13 Pages

Clinical hypertension is a complex disease of the cardiovascular system that can affect the body’s ability to physiologically maintain homeostasis. Blood pressure is measured as systolic pressure of the heart and diastolic pressure. When the sy...

  • Case Report
  • Open Access
2,291 Views
7 Pages

PRKAG2 Variant, Motor Neuron Disease, and Parkinsonism: Fortuitous Association or a Potentially Underestimated Pathophysiological Mechanism?

  • Marco Orsini,
  • Wladimir Bocca Vieira de Rezende Pinto,
  • Paulo Sgobbi and
  • Acary Souza Bulle Oliveira

25 July 2024

A 72-year-old Brazilian woman presented with a 4-year history of rest tremors of the hands, followed by slowness of movement, and a diagnosis of idiopathic Parkinson’s disease. She was started on dopamine agonists with significant improvement....

  • Review
  • Open Access
23 Citations
7,911 Views
24 Pages

Brugada Syndrome: From Molecular Mechanisms and Genetics to Risk Stratification

  • Irene Paula Popa,
  • Dragomir N. Șerban,
  • Minela Aida Mărănducă,
  • Ionela Lăcrămioara Șerban,
  • Bogdan Ionel Tamba and
  • Ionuț Tudorancea

7 February 2023

Brugada syndrome (BrS) is a rare hereditary arrhythmia disorder, with a distinctive ECG pattern, correlated with an increased risk of ventricular arrhythmias and sudden cardiac death (SCD) in young adults. BrS is a complex entity in terms of mechanis...