Pseudo cryomapping for ablation of atrioventricular nodal reentry tachycardia: A single center North American experience.

BACKGROUND: Most literature for cryoablation of atrioventricular nodal reentry tachycardia (AVNRT) is based on -30 degree celsius cryomapping with 4 & 6 mm distal electrode catheters. The cryomapping mode is not available on the 6 mm cryocatheter in the United States. We describe a technique for 'pseudo' mapping at -80° using a 6 mm cryocatheter and report on short and long term outcomes. METHODS: A retrospective analysis of all index cases (n = 253) of cryoablation of AVNRT at a single North American institution during the period of 2003-2010 was performed. The majority of cases utilized a 6 mm distal electrode tip catheter. Long term follow up (2.4 ± 1.8 years) was performed via review of the medical record and by questionnaire or telephone if necessary. RESULTS: Acute ablation success was achieved in 93% of cases, with transient conduction defects noted in 39% of cases, and long term conduction defects in 1.6% of cases (4 patients with PR prolongation, 2 of which were permanent). General anesthesia, male gender and presence of structural heart disease were more common in the acute failure cohort. The recurrence rate for AVNRT was 8%. These patients tended to be younger and had more transient A-V conduction defects during the index procedure than those without a recurrence. CONCLUSIONS: In conclusion, anatomic cryoablation of AVNRT utilizing a 6 mm electrode catheter with mapping performed at -80° Celsius is a safe procedure with good long term efficacy. Transient A-V block during the index procedure increases the risk of late recurrence.

Brugada syndrome in children - Stepping into unchartered territory.

Brugada syndrome (BrS) is an autosomal dominant inherited channelopathy. It is associated with a typical pattern of ST-segment elevation in the precordial leads V1-V3 and potentially lethal ventricular arrhythmias in otherwise healthy patients. It is frequently seen in young Asian males, in whom it has previously been described as sudden unexplained nocturnal death syndrome. Although it typically presents in young adults, it is also known to present in children and infants, especially in the presence of fever. Our understanding of the genetic pathogenesis and management of BrS has grown substantially considering that it has only been 24 years since its first description as a unique clinical entity. However, there remains much to be learned, especially in the pediatric population. This review aims to discuss the epidemiology, genetics, and pathogenesis of BrS. We will also discuss established standards and new innovations in the diagnosis, prognostication, risk stratification, and management of BrS. Literature search was run on the National Center for Biotechnology Information's website, using the Medical Subject Headings (MeSH) database with the search term "Brugada Syndrome" (MeSH), and was run on the PubMed database using the age filter (birth-18 years), yielding 334 results. The abstracts of all these articles were studied, and the articles were categorized and organized. Articles of relevance were read in full. As and where applicable, relevant references and citations from the primary articles were further explored and read in full.

Catecholaminergic polymorphic ventricular tachycardia: An exciting new era.

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a highly malignant inheritable cardiac channelopathy. The past decade and a half has provided exciting new discoveries elucidating the genetic etiology and pathophysiology of CPVT. This review of the current literature on CPVT aims to summarize the state of the art in our understanding of the genetic etiology and the molecular pathogenesis of CPVT, and how these relate to our current approach to diagnosis and management. We will also shed light on groundbreaking new work that will continue to refine the management of CPVT in the future. As our knowledge of CPVT continues to grow, further studies will yield a better understanding of the efficacy and pitfalls of established diagnostic approaches and therapies as well as help shape newer diagnostic and treatment strategies. Two separate searches were run on the National Center for Biotechnology Information's (NCBI) website. The first used the medical subject headings (MeSH) database using the term "catecholaminergic polymorphic ventricular tachycardia" that was run on the PubMed database using the age filter (birth to 18 years), and it yielded 58 results. The second search using the MeSH database with the search term "catecholaminergic polymorphic ventricular tachycardia," applying no filters yielded 178 results. The abstracts of all these articles were studied and the articles were categorized and organized. Articles of relevance were read in full. As and where applicable, relevant references and citations from the primary articles were further explored and read in full.

Inherited arrhythmias: The cardiac channelopathies.

Ion channels in the myocardial cellular membrane are responsible for allowing the cardiac action potential. Genetic abnormalities in these channels can predispose to life-threatening arrhythmias. We discuss the basic science of the cardiac action potential; outline the different clinical entities, including information regarding overlapping diagnoses, touching upon relevant genetics, new innovations in screening, diagnosis, risk stratification, and management. The special considerations of sudden unexplained death and sudden infant death syndrome are discussed. Scientists and clinicians continue to reconcile the rapidly growing body of knowledge regarding the molecular mechanisms and genetics while continuing to improve our understanding of the various clinical entities and their diagnosis and management in clinical setting. Two separate searches were run on the National Center for Biotechnology Information's website. The first using the term cardiac channelopathies was run on the PubMed database using filters for time (published in past 5 years) and age (birth-18 years), yielding 47 results. The second search using the medical subject headings (MeSH) database with the search terms "Long QT Syndrome" (MeSH) and "Short QT Syndrome" (MeSH) and "Brugada Syndrome" (MeSH) and "Catecholaminergic Polymorphic Ventricular Tachycardia" (MeSH), applying the same filters yielded 467 results. The abstracts of these articles were studied, and the articles were categorized and organized. Articles of relevance were read in full. As and where applicable, relevant references and citations from the primary articles where further explored and read in full.

Recent advances in the understanding and management of long QT syndrome.

PURPOSE OF REVIEW: The sudden death of a previously healthy young individual is a dreadful occurrence. Identifying those at risk of such a dire outcome, and appropriately managing and counseling them, has been an ongoing challenge, but rapid advances are being made. This review will focus on the long QT syndrome (LQTS), the most common of the potentially lethal inheritable arrhythmias, with specific focus on the genetics relevant to clinical presentation, therapy and response. RECENT FINDINGS: The past 2 decades have seen tremendous progress in the field of inheritable arrhythmias. Emphasis is given to risk stratification, screening of family members, and the counseling of young athletes, as well as new developments in screening and treatment. SUMMARY: For the primary care provider, long QT syndrome should be considered during the evaluation of syncope, near-syncope and seizures, especially in the setting of exercise or with a family history of sudden unexplained death in a first-degree relative. The corrected QT interval (QTc) should be assessed as a routine when obtaining electrocardiograms. If there are concerns on the basis of electrocardiogram findings, medical history or family history, referral to a cardiologist is indicated. Providers need to be cognizant of the challenges of therapy and lifestyle changes for patients and families with long QT syndrome.

Coronary artery dilation among patients presenting with systemic-onset juvenile idiopathic arthritis.

OBJECTIVE: To evaluate coronary artery diameters among patients presenting with systemic-onset juvenile idiopathic arthritis (SoJIA). METHODS: Fifty cases of SoJIA were reviewed. At the time of initial presentation with fever, 12 patients had echocardiograms that included a complete evaluation of the coronary arteries. A single reviewer measured the diameters of the left main, proximal left anterior descending, and proximal right coronary arteries. Body surface area-adjusted z scores were calculated with respect to a normative population. RESULTS: Coronary artery dilation (z score: >2) was observed for 5 of the 12 patients with SoJIA who had echocardiograms performed at the time of presentation with fever. No patient developed a coronary artery aneurysm, and all of the coronary artery z scores normalized within 4 months. Only 2 of the 5 patients with coronary artery z scores of >2 fulfilled the clinical criteria for Kawasaki disease, the most commonly recognized cause of coronary artery dilation among children. CONCLUSIONS: Children presenting with SoJIA may have coronary artery dilation similar to that observed for children with Kawasaki disease. These data suggest that the presence of coronary artery dilation on initial echocardiograms for patients with fever does not exclude the diagnosis of SoJIA.