Incidence of Atrial Fibrillation After Radiofrequency Catheter Ablation for Atrial Tachycardia in Congenital Heart Disease.

BACKGROUND: Atrial tachycardia (AT) and atrial fibrillation (AF) coexist in 30% of congenital heart disease (CHD) patients. Successful atrial tachycardia catheter ablation (ATCA) might prevent AF. Data on new-onset AF after ATCA in CHD is scarce. OBJECTIVES: This study aimed to evaluate the incidence of new-onset AF after ATCA and to assess clinical characteristics associated with new-onset AF after ATCA in CHD. METHODS: CHD patients referred for ATCA to 3 European centers were included. New occurrence of AF was defined as electrocardiographic documentation of AF after any ATCA procedure in patients without history of AF. RESULTS: In 277 CHD patients (median age 37 years [Q1, Q3: 23, 49 years], 58% men, 59 [21%] simple, 111 [40%] moderate, and 107 [39%] complex CHD), AF occurred in 25 patients (9%) a median of 8 months (Q1, Q3: 4, 27 months) after ATCA. New-onset AF was persistent in the majority of the patients (17 of 25 [63%]). Patients with new-onset AF were older (44 years [Q1, Q3: 29, 55 years] vs 36 years [Q1, Q3: 23, 49 years]; P = 0.009) and more frequently had simple CHD (13 of 25 [52%] vs 46 of 252 [18%], respectively; P < 0.0001). Acute ATCA success rates were similar in patients with and without AF (52% vs 48%; P = 0.429). Simple CHD was an independent predictor of new-onset AF during follow-up. CONCLUSIONS: In our large cohort of patients with congenital heart disease, new-onset AF after ablation for AT occurred in only 9% of the patients. AF occurred without AT recurrence and was persistent in the majority of patients.

Long-term use of everolimus for refractory arrhythmia in a child with tuberous sclerosis complex.

Tuberous sclerosis complex is associated with the occurrence of cardiac rhabdomyomas that may result in life-threatening arrhythmia unresponsive to standard antiarrhythmic therapy. We report the case of an infant with multiple cardiac rhabdomyomas who developed severe refractory supraventricular tachycardia (SVT) that was successfully treated with everolimus. Pharmacological mTOR inhibition rapidly improved arrhythmia within few weeks after treatment initiation and correlated with a reduction in tumor size. Intermediate attempts to discontinue everolimus resulted in rhabdomyoma size rebound and recurrence of arrhythmic episodes, which resolved on resumption of therapy. While everolimus treatment led to successful control of arrhythmia in the first years of life, episodes of SVT reoccurred at the age of 6 years. Electrophysiologic testing confirmed an accessory pathway that was successfully ablated, resulting in freedom of arrhythmic events. In summary we present an in-depth evaluation of the long-term use of everolimus in a child with TSC-associated SVT, including the correlation between drug use and arrhythmia outcome. This case report provides important information on the safety and efficacy of an mTOR inhibitor for the treatment of a potentially life-threatening cardiac disease manifestation in TSC for which the optimal treatment strategy is still not well established.

Anatomy for ablation of atrioventricular nodal reentry tachycardia and accessory pathways.

The atrioventricular (AV) valve plane and the central septum are of particular importance for electrophysiological diagnosis and interventional therapy of supraventricular tachycardias because accessory electrical connections of various types may be present in addition to the specific conduction system. Although modern 3D electroanatomic reconstruction systems including high-density mapping can be of great assistance, detailed knowledge of the anatomic structures involved, their complex three-dimensional arrangement, and their electrical properties in conjunction with electrophysiological features of supraventricular arrhythmias is essential for safe and efficient electrophysiological treatment. The aim of this article is to present current anatomical, topographical, and electrophysiological findings against the background of historical, seminal, and still indispensable literature.

Catheter Ablation of Atrioventricular Nodal Reentrant Tachycardia in Patients With Congenital Heart Disease.

Atrioventricular (AV) nodal reentrant tachycardia represents the most common regular supraventricular arrhythmia in humans, and catheter ablation of the so called slow AV nodal pathway has been effectively performed for decades. In patients with congenital heart disease, a combination of different factors makes catheter ablation of AV nodal reentrant tachycardia substrate particularly challenging, including abnormal venous access to intracardiac structures, abnormal intracardiac anatomy, potentially deviant and often unpredictable sites of the specific conduction system, loss of traditional anatomic landmarks, and congenital cardiac surgery that may complicate the access to the AV nodal area. Published experiences have confirmed the efficacy and the relative safety of such procedures when performed by experts, but the risk of complications, in particular AV block, remains non-negligible. A thorough knowledge and understanding of anatomic and electrical specificities according to underlying phenotype are essential in addressing these complex cases. Considering the major consequences associated with AV block in patients with complex congenital heart disease, particularly those without low risk access for transvenous ventricular pacing (eg, single ventricle physiology or Eisenmenger syndrome), the individual risk-benefit ratio should be carefully evaluated. The decision to defer ablation may be the wisest approach in selected patients with either infrequent or hemodynamically tolerated arrhythmias, or when the location of the AV conduction pathways remains uncertain. This narrative review aims to synthetize existing literature on catheter ablation of AV nodal reentrant tachycardia in congenital heart disease, to present main features of common associated pathologies, and to discuss approaches to mapping and safely ablating the slow AV nodal pathway in challenging cases.

Contemporary Patients With Congenital Heart Disease: Uniform Atrial Tachycardia Substrates Allow for Clear Ablation Endpoints With Improved Long-Term Outcome.

[Figure: see text].

Antiarrhythmic drugs-safety and efficacy during pregnancy.

When deciding on antiarrhythmic drug (AAD) treatment, a thorough knowledge of the physiological adaptation processes that occur during pregnancy and their effect on metabolism and the efficacy of AAD is mandatory. Beyond the desired effects of AAD therapy, side effects can occur in pregnant women. Furthermore, potential harm to fetal development-depending on gestational age-needs to be considered. A thorough evaluation of potential risks opposed to expected benefits for mother and fetus should be carried out before initiation of AAD treatment. Regular maternal echocardiography and fetal sonographic examination during pregnancy under AAD treatment are advisable. If possible, serum concentrations of AAD should be measured on a regular basis. Due to electrolyte and volume imbalances after delivery, maternal monitoring is recommended for approximately 48 h under AAD therapy. Current guidelines are based on almost historic analyses, where AAD were often prescribed for other indications than rhythm disorders. In clinical practice, AAD predominantly used during pregnancy are intravenous adenosine for acute treatment of atrioventricular nodal dependent tachycardias, whereas betablockers, sotalol, and flecainide can be orally administered for long-term therapy.

Therapy of ventricular arrhythmias in patients suffering from isolated left ventricular non-compaction cardiomyopathy.

AIMS: Non-compaction cardiomyopathy (NCCM) is associated with high rates of mortality and morbidity. Knowledge regarding risk stratification, arrhythmogenesis, therapy, and prognosis is limited. The aim of this study was to analyse the outcome of patients suffering from NCCM and ventricular arrhythmias (VAs) focusing on a treatment with implantable cardioverter-defibrillator (ICD) therapy and catheter ablation. METHODS AND RESULTS: We conducted a multicentre observational study on 18 patients with NCCM, who underwent ICD implantation for secondary (n = 12) and primary (n = 6) prevention. In patients with multiple symptomatic episodes of VAs catheter ablation was performed. During a follow-up of 62 ± 42 months, 12 patients (67%) presented with appropriate ICD therapies [ventricular tachycardia (VT): n = 8; ventricular fibrillation (VF): n = 4; VT/VF: n = 3]. Ten patients underwent catheter ablation for VT/VF. Solely endocardial ablation was conducted in eight patients, and in two patients endo- and epicardial ablation was performed within the same procedure. Acute procedural success was achieved in 9/10 patients. Ventricular tachycardia recurrence was observed in two patients and the median arrhythmia free interval was 9.5 months (interquartile range 5.3-21 months). One patient underwent reablation, four patients died due to the underlying NCCM, and one patient received a left ventricular assist device. CONCLUSION: Ventricular arrhythmias are common in patients suffering from NCCM and ICD therapy may be effective for primary and secondary prevention. In our cohort, consisting of patients with multiple VA episodes and recurrent ICD therapy, catheter ablation offered a safe and effective therapeutically option.

[Mapping and ablation of cardiac arrhythmias : Never forget where you are coming from]. / Mapping und Ablation von Herzrhythmusstörungen : "Never forget where you are coming from".

With the rapid development of new mapping and imaging technologies as well as catheter ablation technologies, it is increasingly important to understand the basic concepts of conventional mapping and ablation of cardiac arrhythmias. Prerequisite for successful ablation is the exact identification of the tachycardia mechanism and subsequent localization of the origin or tachycardic substrate. Only intracardiac electrograms provide decisive information regarding activation time and signal morphology. In some arrhythmias, it is necessary to supplement conventional mapping with so-called pace and/or entrainment mapping. This article aims to discuss and demonstrate the fundamentals of intracardiac mapping as it relates to the mapping and ablation of supraventricular and ventricular arrhythmias based on representative clinical cases. Modern three-dimensional mapping methods make it possible to individually optimize established ablation strategies with significantly better spatial resolution. The authors aimed to demonstrate that intracardiac uni- and bipolar electrograms provide essential information about timing and morphology guiding successful catheter ablation. Furthermore, our article provides useful information about conventional cardiac mapping techniques including activation mapping, pace mapping, and individual substrate mapping.

Catheter Ablation for Atrial Fibrillation in Adults With Congenital Heart Disease: Lessons Learned From More Than 10 Years Following a Sequential Ablation Approach.

OBJECTIVES: This study aimed to evaluate the impact, safety, and success of atrial fibrillation (AF) ablation in adults with congenital heart disease (ACHD) transferring ablation strategies established in normal hearts. BACKGROUND: AF is an emerging arrhythmia in ACHD. METHODS: Fifty-seven consecutive ACHD (median age 51.1 ± 14.8 years) with drug-refractory AF were analyzed who underwent catheter ablation between 2004 and 2017. CHD was classified according to its complexity into mild (61.4%), moderate (17.5%), and severe (21.1%) lesions. AF ablation was performed in 104 procedures following a sequential ablation approach. RESULTS: Of the 57 patients, 30 underwent corrective surgery, 6 underwent palliative surgery, 5 had catheter interventions, and 16 were natural survivors. Follow-up was available for all patients (median 41 ± 36 months). The median duration of cyanosis was 9.2 ± 19.7 years, and the time of volume or pressure overload prior to corrective surgery or intervention was 26.1 ± 21.2 years and 18.1 ± 15.8 years, respectively. The Kaplan-Meier estimate for arrhythmia-free survival following the index ablation procedure was 63% for 1 year and 22% for 5 years. Performing subsequent ablation procedures (2.0 ± 0.5), the Kaplan-Meier estimate significantly improved, with 99% for 1 year and 83% for 5 years (p < 0.01). Five patients died during follow-up due to their underlying CHD condition or underwent transplantation. CONCLUSIONS: AF ablation strategies established in normal hearts can be transferred to ACHD. The treatment is safe and effective with acceptable long-term results. Varying anatomical pre-conditions and the heterogeneous population itself are challenging and contribute toward a higher reablation rate. Therefore, AF ablation in ACHD should be reserved for dedicated and highly specialized teams.

Value of an old school approach: safety and long-term success of radiofrequency current catheter ablation of atrioventricular nodal reentrant tachycardia in children and young adolescents.

PURPOSE: Radiofrequency current energy (RFC) ablation is still considered as the gold standard for atrioventricular nodal reentrant tachycardia (AVNRT). Success-rates for AVNRT ablation vary irrespective of the ablation technology and strategy. This study aimed to access safety, efficacy, and long-term outcome of RFC catheter ablation for the treatment of AVNRT in children and adolescents aged < 19 years with special focus on modulation versus ablation of the AV nodal slow pathway (SP). METHODS: A total number of 1143 patients (pts) < 19 years were referred for invasive electrophysiological testing due to paroxysmal supraventricular tachycardia (SVT). RESULTS: Diagnosis of AVNRT was confirmed in 412 pts, and RFC-guided ablation was attempted in 386 pts (age 13.0 ± 3.5 years). No permanent complications were observed. RFC application resulted in SP-ablation in 171/386 (44.3%) and in SP modulation in 208/386 (53.9%) children, whereas attempts for RFC treatment failed in 7 pts. Follow-up was completed for 396/412 patients (96.1%). Within a mean follow-up period of 54.9 ± 39.7 months, in 51/379 pts (13.5%) AVNRT recurrence was observed. The median time until tachycardia recurrence was 19.5 months. No difference for AVNRT recurrence was found comparing SP ablation versus SP modulation (p > 0.05), whereas the recurrence rate was significantly higher in patients with non-inducible SVT and therefore empiric SP treatment as compared to patients with inducible AVNRT (p = 0.01). CONCLUSIONS: RFC-guided ablation for AVNRT in children and adolescents is safe and leads to an acceptable long-term freedom from recurrences. SP modulation and SP ablation resulted in comparable acute and long-term success rates. Late AVNRT recurrences can occur even after years of freedom from tachycardia-related symptoms.

Arrhythmias in congenital heart disease: a position paper of the European Heart Rhythm Association (EHRA), Association for European Paediatric and Congenital Cardiology (AEPC), and the European Society of Cardiology (ESC) Working Group on Grown-up Congenital heart disease, endorsed by HRS, PACES, APHRS, and SOLAECE.

The population of patients with congenital heart disease (CHD) is continuously increasing with more and more patients reaching adulthood. A significant portion of these young adults will suffer from arrhythmias due to the underlying congenital heart defect itself or as a sequela of interventional or surgical treatment. The medical community will encounter an increasing challenge as even most of the individuals with complex congenital heart defects nowadays become young adults. Within the past 20 years, management of patients with arrhythmias has gained remarkable progress including pharmacological treatment, catheter ablation, and device therapy. Catheter ablation in patients with CHD has paralleled the advances of this technology in pediatric and adult patients with structurally normal hearts. Growing experience and introduction of new techniques like the 3D mapping systems into clinical practice have been particularly beneficial for this growing population of patients with abnormal cardiac anatomy and physiology. Finally, device therapies allowing maintanence of chronotropic competence and AV conduction, improving haemodynamics by cardiac resynchronization, and preventing sudden death are increasingly used. For pharmacological therapy, ablation procedures, and device therapy decision making requires a deep understanding of the individual pathological anatomy and physiology as well as detailed knowledge on natural history and long-term prognosis of our patients. Composing expert opinions from cardiology and paediatric cardiology as well as from non-invasive and invasive electrophysiology this position paper was designed to state the art in management of young individuals with congenital heart defects and arrhythmias.

[Non-invasive treatment of tachycardias during childhood]. / Nichtinvasives Management von tachykarden Rhythmusstörungen bei Kindern.

In principle tachycardias during childhood do not differ from those in adulthood but they present with a significant age-dependency. Additionally the clinical presentation has a broad spectrum related to the different ages, from the neonatal period until adolescence. If congenital heart disease is present the hemodynamic compromise may be accentuated. This paper describes the diagnostic and therapeutic approaches to pediatric tachycardias with focus upon the age dependent aspects and the presense of congenital heart disease, either native or postoperative.

[Interventional treatment of tachyarrhythmia in children with congenital heart disease]. / Interventionelle Therapie von tachykarden Herzrhythmusstörungen bei Kindern mit angeborenem Herzfehler.

In children and adolescents with congenital heart disease (CHD) tachyarrhythmia occurs more frequently compared to patients with otherwise normal hearts. Arrhythmia substrates may be a natural part of certain congenital cardiac malformations or may result from long lasting myocardial deterioration as a result of CHD and/or cardiac surgery. Treatment of tachycardia is more frequently required even in early childhood, as the impact on quality of life, morbidity and mortality is higher due to an often reduced hemodynamic tolerance. Over the past 20 years interventional electrophysiology has been established as the therapy of choice for the majority of chronic or chronically recurrent tachycardia even in children with CHD. The success and risks of treatment are predominantly influenced by the individual expression of the cardiac anomaly and, if surgery has been performed, the highly variant postoperative anatomy. Introduction of 3D electroanatomical mapping systems together with modern cardiac imaging tools have significantly contributed to an improved understanding, particularly in postoperative tachycardia. Despite such progress, success rates are lower and recurrences are more frequent compared to patients without CHD. Complex and often multiple tachycardia courses account for the still limited performance as well as a frequently insufficient lesion formation with the use of radiofrequency current in the hypertrophic and fibrotic myocardium. Electrophysiology in children and adolescents, particularly if CHD is present, represents a highly specialized discipline requiring a high expertise in CHD, CHD surgery and cardiac electrophysiology and is ideally imbedded within an interdisciplinary cardiological and cardiosurgical setting.

Pharmacological and non-pharmacological therapy for arrhythmias in the pediatric population: EHRA and AEPC-Arrhythmia Working Group joint consensus statement.

In children with structurally normal hearts, the mechanisms of arrhythmias are usually the same as in the adult patient. Some arrhythmias are particularly associated with young age and very rarely seen in adult patients. Arrhythmias in structural heart disease may be associated either with the underlying abnormality or result from surgical intervention. Chronic haemodynamic stress of congenital heart disease (CHD) might create an electrophysiological and anatomic substrate highly favourable for re-entrant arrhythmias. As a general rule, prescription of antiarrhythmic drugs requires a clear diagnosis with electrocardiographic documentation of a given arrhythmia. Risk-benefit analysis of drug therapy should be considered when facing an arrhythmia in a child. Prophylactic antiarrhythmic drug therapy is given only to protect the child from recurrent supraventricular tachycardia during this time span until the disease will eventually cease spontaneously. In the last decades, radiofrequency catheter ablation is progressively used as curative therapy for tachyarrhythmias in children and patients with or without CHD. Even in young children, procedures can be performed with high success rates and low complication rates as shown by several retrospective and prospective paediatric multi-centre studies. Three-dimensional mapping and non-fluoroscopic navigation techniques and enhanced catheter technology have further improved safety and efficacy even in CHD patients with complex arrhythmias. During last decades, cardiac devices (pacemakers and implantable cardiac defibrillator) have developed rapidly. The pacing generator size has diminished and the pacing leads have become progressively thinner. These developments have made application of cardiac pacing in children easier although no dedicated paediatric pacing systems exist.