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July 2nd

  • Developmental Basis for Electrophysiol ogical Heterogeneity in the Ventricular and Outflow Tract Myocardium As a Substrate for Life-Threateni ng Ventricular Arrhythmias -- Boukens et al. 104 (1): 19 -- Circulation Research: Reentry is the main mechanism of life-threateni ng ventricular arrhythmias, including ventricular fibrillation and tachycardia. Its occurrence depends on the simultaneous presence of an arrhythmogenic substrate (a preexisting condition) and a "trigger, " and is favored by electrophysiol ogical heterogeneitie s. In the adult heart, electrophysiol ogical heterogeneitie s of the ventricle exist along the apicobasal, left?right, and transmural axes. Also, conduction is preferentially slowed in the right ventricular outflow tract, especially during pharmacologica l sodium channel blockade. We propose that the origin of electrophysiol ogical heterogeneitie s of the adult heart lies in early heart development. The heart is formed from several progenitor regions: the first heart field predominantly forms the left ventricle, whereas the second heart field forms the right ventricle and outflow tract. Furthermore, the embryonic outflow tract consists of slowly conducting tissue until it is incorporated in
  • Molecular Basis of Arrhythmias -- Shah et al. 112 (16): 2517 -- Circulation: The characterizati on of single gene disorders has provided important insights into the molecular pathogenesis of cardiac arrhythmias. Primary electricalal diseases including long-QT syndrome, short-QT syndrome, Brugada syndrome, and catecholaminer gic polymorphic ventricular tachycardia have been associated with mutations in a variety of ion channel subunit genes that promote arrhythmogenes is. Pathological remodeling of ionic currents and network properties of the heart critical for normal electrical propagation plays a critical role in the initiation and maintenance of acquired arrhythmias. This review focuses on the molecular and cellular basis of electrical activity in the heart under normal and pathophysiolog ical conditions to provide insights into the fundamental mechanisms of inherited and acquired cardiac arrhythmias. Improved understanding of the basic biology of cardiac arrhythmias holds the promise of identifying new molecular targets for the treatment of cardiac arrhythmias.
  • Inherited Arrhythmogenic Diseases: The Complexity Beyond Monogenic Disorders -- Priori 94 (2): 140 -- Circulation Research: Twelve years after the identification of the molecular bases of the long-QT syndrome, it is now possible to express some considerations on the impact that genetic findings have had in the understanding of inherited arrhythmogenic diseases. Along with the excitement for the emerging data on genotype/pheno type correlation and for the development of the first recommendation s for gene-specific management of patients, it is also important to acknowledge the unexpected complexity that has emerged. The focus of this article is to analyze the elusive aspects of the relationship between genetic defects and clinical manifestations and to propose some research directions that may provide the needed answers to move forward in the understanding of the genetics of heart rhythm abnormalities.

July 1st

  • MedicalMommas. com Moment: SVT: Dr. John Clark, Akron Children' s Hospital pediatric cardiologist, discusses symptoms and solutions for Superventricul ar Tachycardia (SVT) on MedicalMommas. com.
  • MedicalMommas. com AskMe: SVT: MedicalMommas. com co-founder, Diana Keough chats with Dr. Grace Smith, Akron Children' s Hospital pediatric cardiologist, about kids' heart conditions.
  • MedicalMommas. com MyStory: Diana and Bret Keough: MedicalMommas. com Editor-in-Chie f, Diana Keogh, and her son, Bret, share about how they dealt with Bret's SVT diagnosis and surgery.
  • It's Kind of a Big Deal: Bret Keough, who was diagnosed with SVT three years ago, shares his experience with the diagnosis and surgery. He also offers tips to parents and kids in similar situations.
  • When a Medical Story Hits Home: Medical Journalist and Editor-in-Chie f of MedicalMommas. com, Diana Keough, shares about when her son was diagnosed with Supraventricul ar Tachycardia and had to undergo heart surgery at 15 years old.

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