Management and outcomes of atrial fibrillation in 241 healthy children and young adults: Revisiting "lone" atrial fibrillation-A multi-institutional PACES collaborative study.

BACKGROUND: Atrial fibrillation (AF) in healthy children and young adults is rare. Risk of recurrence and treatment efficacy are not well defined. OBJECTIVE: The purpose of this study was to assess recurrence patterns and treatment efficacy in AF. METHODS: A retrospective multicenter cohort study including 13 congenital heart centers was facilitated by the Pediatric & Congenital Electrophysiology Society (PACES). Patients ≤21 years of age with documented AF from January 2004 to December 2018 were included. Demographics, family and clinical history, medications, electrophysiological study parameters, and outcomes related to the treatment of AF were recorded and analyzed. Patients with contributory diseases were excluded. RESULTS: In 241 subjects (83% male; mean age at onset 16 years), AF recurred in 94 patients (39%) during 2.1 ± 2.6 years of follow-up. In multivariable analysis, predictors of AF recurrence were family history in a first-degree relative <50 years of age (odds ratio [OR] 1.9; P = .047) and longer PR interval in sinus rhythm (OR 1.1 per 10 ms; P = .037). AF recurrence was similar whether patients began no treatment (39/125 [31%]), began daily antiarrhythmic therapy (24/63 [38%]), or had an ablation at any time (14/53 [26%]; P = .39). Ablating non-AF substrate with supraventricular tachycardia improved freedom from AF recurrence (P = .013). CONCLUSION: Recurrence of AF in the pediatric population is common, and the incidence of recurrence was not impacted by "no treatment," "medication only," or "ablation" treatment strategy. Ablation of pathways and other reentrant targets was the only intervention that decreased AF recurrence in children and young adults.

Interrelation between the rs2200733 polymorphism of the ATFB5 gene and atrial fibrillation in Uzbek patients.

OBJECTIVE: Atrial fibrillation (AF) is one of the most common cardiac arrhythmias and a major predictor of morbidity and mortality. AF is a polygenic and polyetiological disease. In various ethnic groups, the strongest and most independent relationship with the development of AF was found with the 4q25 locus, where the ATFB5 gene is located. An analysis of the literature data showed that the carriage of the TT genotype of the rs2200733 ATFB5 gene polymorphism is the most unfavorable genotype for the development of AF. The purpose of the study was to identify the prevalence of genotypes and alleles of the rs2200733 polymorphism of the ATFB5 gene in Uzbek patients with AF. METHODS: The study included 69 Uzbek patients with paroxysmal (n=20) and persistent AF (n=49). The control group (n=30) was composed of Uzbek patients without AF. Genotyping for the carriage of allelic variants of the rs2200733 polymorphism of the ATFB5 gene was performed using the Polymerase Chain Reaction-Restriction Length Polymorphism (PCR-RFLP) method. The distribution of the C and T alleles and the CC, CT, and TT genotypes of the rs2200733 polymorphism of the ATFB5 gene in patients with AF and controls were compared. RESULTS: After genotyping 69 patients with AF, the following distribution of the ATFB5 gene polymorphism rs2200733 was revealed: the CC genotype was detected in 35 (50.72%) patients, the CT genotype in 25 (36.23%) patients, and the TT genotype in 9 (13.05%) patients (p<0.001, χ²=22.435). Moreover, the C allele was detected in 95 (68.8%) patients, and the T allele was detected in 43 (31.2%) patients (p<0.001, χ²=37.696). The distribution of genotypes in the control group was as follows: the CC genotype was detected in 17 individuals (56.7%), the CT genotype was detected in 12 individuals (40%), and the TT genotype was detected in 1 individual (3.3%) (p<0.001, χ²=20.100). Moreover, the C allele was detected in 46 (76.7%) patients, and the T allele was detected in 14 (23.3%) patients (p<0.001, χ²=32.033). The TT genotype of the ATFB5 gene was found to be significantly more prevalent in patients with AF than in controls (13.1% vs 3.3%, p=0.0001). CONCLUSION: The TT genotype of the rs2200733 polymorphism of the ATFB5 gene was found to be significantly more prevalent in Uzbek patients with AF than in controls.

Nonatrial Fibrillation Patients With Complete P Wave Disappearance: An Overlooked Population With High Stroke Risk.

BACKGROUND AND PURPOSE: Complete P wave disappearance (CPWD) in patients without atrial fibrillation is an uncommon clinical phenomenon. We aimed to study the relationship between CPWD and thromboembolism. METHODS: Between July 2007 and December 2018, consecutive patients with CPWD on surface ECG and 24-hour Holter recording were recruited into the study from 4 centers in China. All recruited patients underwent transesophageal echocardiography or cardiac computed tomography to screen for atrial thrombus. Atrial electrical activity and scar were assessed by electrophysiological study (EPS) and 3-dimensional electroanatomic mapping. Cardiac structure and function were assessed by multimodality cardiac imaging. RESULTS: Twenty-three consecutive patients (8 male; mean age 48.5±14.7 years) with CPWD were included. Only 3 patients demonstrated complete atrial electrical silence with atrial noncapture. Thirteen patients who had invasive atrial endocardial mapping demonstrated extensive scar. Pulse-wave mitral inflow Doppler demonstrated absent and dampened A waves in 18 and 5 patients, respectively. Pulse-wave tricuspid inflow Doppler showed absent and dampened A waves in 19 and 4 patients, respectively. Upon recruitment, 8 patients had previous stroke and 3 patients had atrial thrombus. Warfarin was prescribed to all patients. During median follow-up of 42.0 months, 2 patients developed massive ischemic stroke due to warfarin discontinuation. CONCLUSIONS: Our study suggested that CPWD reflects extensive atrial electrical silence and significantly impaired atrial mechanical function. It was strongly associated with thromboembolism and the clinical triad of CPWD-atrial paralysis-stroke was proposed. Anticoagulation should be recommended in such patients.

Fístula coronário-cavitária da artéria coronária circunflexa para o átrio esquerdo como provável etiologia de insuficiência cardíaca: relato de caso / Coronary­cavitary fistula of the circumflex coronary artery to the left atrium as a probable etiology of heart failure: a case report

Fístulas de artérias coronárias têm incidência baixa, sendo diagnosticadas mais frequentemente pelo ecocardiograma ou angiotomografia coronariana, embora a cineangiocoronariografia seja o método padrão-ouro. Têm origem mais comumente na artéria coronária direita, sendo a drenagem para câmaras de baixa pressão o mais habitual. O tratamento pode ser expectante, cirúrgico ou percutâneo. Este relato descreve o caso de uma rara apresentação de insuficiência cardíaca, dada por fístula da coronária circunflexa com drenagem para o átrio esquerdo. (AU)

Coronary artery fistulas have a low incidence and are often diagnosed by echocardiography or coronary computed tomography angiography, although coronary angiography is the gold standard. They commonly originate in the right coronary artery, with drainage to low-pressure chambers being the most frequent finding. Treatment can be expectant, surgical, or percutaneous. This report describes a case of a rare presentation of heart failure due to a fistula of the circumflex coronary artery with drainage into the left atrium. (AU)

Impact of Supraventricular Tachyarrhythmia in Patients With Inherited Cardiac Arrhythmia.

Supraventricular tachyarrhythmia (SVT), especially atrial fibrillation (AF), has been observed in patients with inherited cardiac arrhythmia (ICA). Data on the time course of SVT and the occurrence of SVT other than AF is limited. In this study, we examined the prevalence, co-existence, and the time course of different types of SVT in patients with various ICAs. In this retrospective study, we selected 393 patients (median 49 years, range 17 to 87, 57% male) from a cohort of patients visiting the outpatient clinic for cardiogenetic screening of ICA. Patients' medical records were examined for the occurrence of AF and other SVT. AF/SVT was found in 49 patients (12%, 31 male, 42 ± 17 years). Patients presenting with only AF (n = 12, 3%) were older than patients presenting with only SVT (n = 28, 7%), respectively 52 ± 18 versus 37 ± 14, p = 0.007. Nineteen patients (5%) had multiple episodes of either AF (n = 7, 2%) or SVT (n = 12, 3%). Alternating episodes of AF and SVT occurred in 9 patients (2%). Intervals between second and third AF episodes were significantly shorter than between first and second episodes (p = 0.02). An implantable cardioverter defibrillator (ICD) was implanted in 158 patients (40.2%) and 26 patients (16%) had inappropriate ICD shocks (SVT 25, AF 1), particularly those with multiple SVT episodes (p = 0.003). In patients with a variety of ICAs, episodes of AF/SVT occurred in 12%. In patients with multiple AF episodes, intervals between consecutive episodes became significantly shorter over time. AF/SVT episodes are associated with inappropriate ICD shocks and aggressive therapy of AF/SVT is therefore justified.

Indicaciones de rivaroxabán en diferentes situaciones clínicas / Indications of rivaroxabán in distinct clinical situations

Rivaroxabán es un inhibidor oral directo y altamente selectivo del factor Xa de la coagulación. Los principales resultados del ROCKET-AF mostraron que en pacientes de alto riesgo con fibrilación auricular no valvular, rivaroxabán fue no inferior a la warfarina en la prevención del ictus o embolia sistémica, pero con un menor riesgo de intracraneales, fatales y en órgano crítico. Rivaroxabán ha demostrado ser eficaz y seguro en pacientes con insuficiencia renal, en pacientes ancianos, así como en aquellos con cardiopatía isquémica o ictus previo. Rivaroxabán se toma una sola vez al día. En comparación con los otros nuevos anticoagulantes orales (ACO), rivaroxabán puede proporcionar un mejor cumplimiento y adherencia al tratamiento y, en consecuencia, reducir el riesgo de ictus durante el seguimiento. Además, las interacciones con otros fármacos son escasas. Esto, junto con que es de una sola toma diaria, hace que rivaroxabán suponga una excelente alternativa para la prevención de los episodios tromboembólicos en los pacientes polimedicados con fibrilación auricular. En este manuscrito se revisa la evidencia disponible acerca de la eficacia y seguridad de rivaroxabán en los pacientes con fibrilación auricular en diferentes escenarios clínicos (AU)

Rivaroxaban is an oral highly selective direct factor Xa inhibitor. The main results of ROCKET-AF showed that in high risk patients with nonvalvular atrial fibrillation, rivaroxabán was noninferior to warfarin for the prevention of stroke or systemic embolism, but with lesser risk of intracranial and fatal bleeding. Remarkably, rivaroxaban has been proven to be effective and safe in patients with renal dysfunction, elderly patients as well as in those with ischemic heart disease or previous stroke . Rivaroxaban is taken only once daily. Compared with other new oral anticoagulants, this may provide better compliance and medication adherence and, consequently, a reduction in the risk of stroke during the follow-up. Moreover, the interactions with other drugs are low. This together with the once daily dose makes rivaroxabán an excellent alternative for the prevention of thromboembolic events in polymedicated patients with atrial fibrillation. This manuscript reviews the available evidence about the efficacy and safety of rivaroxabán in patients with atrial fibrillation in different clinical settings (AU)

Evidencias sobre el coste económico de los nuevos anticoagulantes / Evidence on the economic cost of the new anticoagulants

Con la aprobación de los nuevos fármacos anticoagulantes, con un precio superior a los clásicos fármacos antivitamina K, se hace necesario realizar un análisis de costeefectividad de estos nuevos fármacos, especialmente en el contexto de crisis económica en que estamos inmersos. Se hace una búsqueda en PubMed de los estudios publicados sobre coste-efectividad de los nuevos anticoagulantes. Todos los estudios mostrados utilizan una metodología común, un modelo de Markov que simula la historia natural de la enfermedad para distintas cohortes de pacientes en varios sistemas de salud, nuestro sistema público de salud español, así como de varios países de nuestro entorno. En este modelo se compara el coste-efectividad de los nuevos anticoagulantes con la warfarina. Hemos encontrado, en la literatura médica reciente, varios estudios para cada uno de los nuevos anticoagulantes, donde se analizan los costes totales, directos e indirectos, los costes por número de episodios, los años de calidad de vida ajustados, los costes de seguimiento, la razón de coste-efectividad incremental. Desde el punto de vista de coste-efectividad, los resultados son favorables para estos fármacos en distintos escenarios frente a la práctica clínica habitual en estos sistemas de salud. En estos modelos, los nuevos anticoagulantes se muestran como fármacos coste-efectivos frente a los clásicos antivitamina K, tanto de forma global como en algunos subgrupos. Corresponde a nuestras autoridades sanitarias establecer grupos de pacientes prioritarios para estos nuevos anticoagulantes y establecer un uso racional de éstos dentro del Sistema Nacional de Salud (AU)

With the adoption of new anticoagulant drugs, with a higher price than the classical drugs antivitamina K, it's necessary to perform an analysis of cost-effectiveness of these new drugs, especially in the context of economic crisis in which we are immersed. We have performed a a search on PubMed of the published studies on cost-effectiveness of new anticoagulants. All the listed studies used a common methodology, a Markov model that simulates the natural history of the disease for different cohorts of patients in various health systems, our public health system in Spanish, as well as several neighbouring countries. This model compares the cost-effectiveness of the new anticoagulants with warfarin. We have found recent medical literature studies for each one of new anticoagulants, where total, direct and indirect costs, costs per number of events, quality of life years adjusted, follow-up costs, incremental cost-effectiveness ratios were analized. The results about cost-effectiveness are favourable for these new drugs in different scenarios in comparison with the common practice in these public systems of health. In these models, the new anticoagulants are shown as drugs cost-effective against the drugs antivitamina K globally and in some subgroups. It corresponds to our health authorities to establish groups of priority patients for these new anticoagulants and a rational use of them within the national health system (AU)

Sudden cardiac death in pediatrics.

PURPOSE OF REVIEW: Sudden cardiac death (SCD) is a rare but tragic event in children. This review highlights the important advances in this field during the last year, both in the understanding of the underlying diseases and in improvements in the management of patients at risk for SCD. RECENT FINDINGS: The study of SCD has focused on several major themes: progress in understanding the causes and treatments of genetic cardiovascular diseases, improved management of patients with congenital heart disease with SCD risk, treatment strategies for SCD risk reduction in patients with cardiomyopathies, and defining guidelines for athletic preparticipation screening. SUMMARY: With advances in understanding of the causes and risk factors for SCD, management of children at risk for SCD continues to improve. This includes improved clinical criteria for identifying SCD risk as well as optimizing management strategies such as exercise restriction, antiarrhythmic medications, and implantable cardioverter defibrillator therapy.

Sinus node function in children with congenital complete atrioventricular block.

AIMS: Children with congenital complete atrioventricular block (CCAVB) often need pacemaker therapy. In these children, it may be preferable to use single-lead VDD pacing, but for VDD pacing a normal sinus node function is required. Our aim was to study sinus node function in children with CCAVB. METHODS AND RESULTS: We longitudinally evaluated sinus rate in 36 children with CCAVB and normal anatomy of the heart. The rate of sinus rhythm on a 12-lead ECG, in Holter recordings, and exercise tests were evaluated at regular intervals. Age at the first visit of the children was 2.5+/-3.3 years (mean+/-SD). Follow-up was 10.6+/-7.3 years. The rate of sinus rhythm on a 12-lead ECG was at every age within the normal values for age (e.g. 0-1 year: 153+/-24 bpm, and 17-18 years: 76+/-4 bpm). Lowest and highest sinus rates in the Holter recordings were normal. During exercise, mean sinus rate in the total group of children increased from 92+/-8 at rest to 171+/-9 bpm at maximal exercise. CONCLUSION: We conclude that sinus node function is normal in children with CCAVB. Because of the normal increase in sinus rate during exercise, a single-lead VDD pacemaker can be safely implanted in these children.

Genome-wide linkage scan identifies a novel genetic locus on chromosome 5p13 for neonatal atrial fibrillation associated with sudden death and variable cardiomyopathy.

BACKGROUND: Atrial fibrillation (AF) is the most common sustained cardiac arrhythmia, and patients with AF have a significantly increased risk for ischemic stroke. Approximately 15% of all strokes are caused by AF. The molecular basis and underlying mechanisms and pathophysiology of AF remain largely unknown. METHODS AND RESULTS: We have identified a large AF family with an autosomal recessive inheritance pattern. The AF in the family manifests with early onset at the fetal stage and is associated with neonatal sudden death and, in some cases, ventricular tachyarrhythmias and waxing and waning cardiomyopathy. Genome-wide linkage analysis was performed for 36 family members and generated a 2-point logarithm of the odds (LOD) score of 3.05 for marker D5S455. The maximum multipoint LOD score of 4.10 was obtained for 4 markers: D5S426, D5S493, D5S455, and D5S1998. Heterozygous carriers have significant prolongation of P-wave duration on ECGs compared with noncarriers (107 versus 85 ms on average; P=0.000012), but no differences between these 2 groups were detected for the PR interval, QRS complex, ST-segment duration, T-wave duration, QTc, and R-R interval (P>0.05). CONCLUSIONS: Our findings demonstrate that AF can be inherited as an autosomal recessive trait and define a novel genetic locus for AF on chromosome 5p13 (arAF1). A genetic link between AF and prolonged P-wave duration was identified. This study provides a framework for the ultimate cloning of the arAF1 gene, which will increase the understanding of the fundamental molecular mechanisms of atrial fibrillation.

[Noncompaction of the left ventricular myocardium. Case report and review of the literature]. / Noncompaction des linksventrikulären Myokards. Fallbericht und Literaturübersicht.

Left ventricular non-compaction is an unclassified cardiomyopathy characterized by an excessively prominent trabecular meshwork due to an arrest in myocardial morphogenesis. We report on a 51-year-old female patient with abnormal myocardial trabeculations associated with a congenital mitral valve stenosis post commissurotomy at the age of 14. In the present case report, clinical manifestation of the disorder included impaired left ventricular systolic function and atrial fibrillation. Noncompaction was diagnosed by echocardiography and levocardiography. In addition, a review of the literature on this rare disorder is presented.

Congenital malformations of the right atrium and the coronary sinus: an analysis based on 103 cases reported in the literature and two additional cases.

STUDY OBJECTIVES: Congenital malformations of the right atrium (RA) and the coronary sinus (CS) are rare, and only sporadic cases have been reported. Little is known about the clinical relevance of this disorder. We report on two patients, one with a giant RA diverticulum, the other with a diverticulum of the CS, and review 103 cases of such malformations that have been reported previously. DESIGN: A MEDLINE search was performed to collect all cases of congenital malformations of the RA and the CS reported in the literature between 1955 and 1998. Cases were classified into the following categories: (1) congenital enlargement of the RA; (2) single diverticulum of the RA; (3) multiple diverticula of the RA; and (4) diverticulum of the CS. Clinical presentation and outcome of the different types of malformations were analyzed. RESULTS: The patients most frequently presenting with symptoms were those with diverticula of the CS (n = 28) followed by those with single diverticula of the RA (n = 13), multiple diverticula (n = 4), and congenital enlargements of the RA (n = 60). The percentages of symptomatic patients were 93, 84, 75, and 53%, respectively. Symptoms were frequently caused by arrhythmias. Supraventricular tachycardia (SVT) was found in 42 of the patients (40%) and was most common in patients with diverticula of the CS (24 of 28 patients) and multiple atrial diverticula (3 of 4 patients). Sudden cardiac death was reported more frequently in patients with diverticula of the CS (18%) compared to those with congenital enlargement of the RA (5%) or single or multiple diverticula of the RA (6%). All seven patients with diverticula of the CS who were not treated with catheter or surgical ablation eventually died. CONCLUSION: Congenital malformations of the RA and the CS frequently are associated with arrhythmias. SVT and sudden cardiac death have been reported in a significant percentage of patients with diverticula of the CS.

Left isomerism in a middle-aged woman with early-onset atrial fibrillation.

An unusual case of left isomerism in a 52-year-old woman with early-onset atrial fibrillation was presented. Magnetic resonance imaging clearly delineated the morphology of both atrial appendages, the vascular anatomy and all other associated anomalies, obviating further invasive studies. The discovery of left isomerism at advanced age indicates that abnormal atrial situs itself is of less clinical importance, and despite the associated sinus node dysfunction may predispose the heart to atrial fibrillation.

Surgical treatment of the Wolff-Parkinson-White syndrome in children.

From 1986 to 1989, seven children ranging in age from 5 months to 16 years underwent surgical treatment for the Wolff-Parkinson-White syndrome at the Shiga University of Medical Science. None of the patients had any other associated congenital heart disease. There was a right free wall accessory pathway in four patients and a left free wall accessory pathway in three. Surgical ablation of these accessory pathways was performed on eight occasions, using the endocardial approach three times and the epicardial approach five. All the children are alive and none has since had episodes of tachycardia. Only one patient had a recurrent delta wave, which was noted 18 months after the operation. Surgical ablation of the accessory pathway for the Wolff-Parkinson-White syndrome can be performed safely, even in infants and children; it is concluded that this useful procedure is capable of improving a patient's quality of life.

Intrauterine and postnatal atrial fibrillation in the Wolff-Parkinson-White syndrome.

A fetal tachyarrhythmia was discovered at the thirty-second week of gestation of a 22-year old woman. Fetal echocardiography revealed atrial fibrillation with rapid ventricular rate, without any other demonstrable cardiac abnormality. In spite of therapeutic maternal blood levels of digoxin, the fetal ventricular rate and cardiac size increased, which prompted us to perform cesarean section at the thirty-fourth week of gestation. A baby with a Wolff-Parkinson-White syndrome but no other cardiac anomaly was delivered. Recurrent episodes of nonsustained atrial fibrillation with conduction over the accessory pathway occurred in the first hours of life. The Wolff-Parkinson-White pattern was not present on subsequent ECG recordings. The use of echocardiography in the diagnosis and management of this rare fetal tachyarrhythmia is emphasized.