Andreas Pflaumer

Cryoablation Versus Radiofrequency Energy for the Ablation of Atrioventricular Nodal Reentrant Tachycardia (the CYRANO Study) Results From a Large Multicenter Prospective Randomized Trial

Background— Cryoablation has emerged as an alternative to radiofrequency catheter ablation (RFCA) for the treatment of atrioventricular (AV) nodal reentrant tachycardia (AVNRT). The purpose of this prospective randomized study was to test whether cryoablation is as effective as RFCA during both short-term and long-term follow-up with a lower risk of permanent AV block. Methods and Results— A total of 509 patients underwent slow pathway cryoablation (n=251) or RFCA (n=258). The primary end point was immediate ablation failure, permanent AV block, and AVNRT recurrence during a 6-month follow-up. Secondary end points included procedural parameters, device functionality, and pain perception. Significantly more patients in the cryoablation group than the RFCA group reached the primary end point (12.6% versus 6.3%; P=0.018). Whereas immediate ablation success (96.8% versus 98.4%) and occurrence of permanent AV block (0% versus 0.4%) did not differ, AVNRT recurrence was significantly more frequent in the cryoablation group (9.4% versus 4.4%; P=0.029). In the cryoablation group, procedure duration was longer (138±54 versus 123±48 minutes; P=0.0012) and more device problems occurred (13 versus 2 patients; P=0.033). Pain perception was lower in the cryoablation group (P<0.001). Conclusions— Cryoablation for AVNRT is as effective as RFCA over the short term but is associated with a higher recurrence rate at the 6-month follow-up. The risk of permanent AV block does not differ significantly between cryoablation and RFCA. The potential benefits of cryoenergy relative to ablation safety and pain perception are counterbalanced by longer procedure times, more device problems, and a high recurrence rate. Clinical Trial Registration— URL: http://www.clinicaltrials.gov. Unique identifier: NCT00196222.

Acute Effects and Long-Term Outcome of Pulmonary Vein Isolation in Combination With Electrogram-Guided Substrate Ablation for Persistent Atrial Fibrillation

Complex fractionated atrial electrographic (CFAE) catheter ablation is a new approach for the treatment of atrial fibrillation (AF). It is unclear if acute results of this approach correspond to long-term outcome. The purpose of this study was to prospectively assess acute and long-term successes of an ablation approach combining pulmonary vein isolation (PVI) and ablation of CFAE areas for treatment of persistent AF. PVI and ablation of CFAE areas were performed in 35 patients with persistent AF (30 men, 57+/-9 years of age). At the end of the ablation procedure AF had terminated in 23 of 35 patients (66%) by conversion to sinus rhythm (8 of 23 patients, 35%) or organization to atrial tachycardia (15 of 23 patients, 65%). AF persisted in 12 of 35 patients (34%). At the end of the follow-up period (19+/-12 months), sinus rhythm was present in 26 of 35 patients (74%), including 9 patients with a repeat procedure. This group of 26 patients consisted of 7 of 8 patients (88%) with acute sinus rhythm after the first ablation, 11 of 15 patients (73%) with organization, and 8 of 12 patients (66%) with ongoing AF (p=0.32). In conclusion, a combined approach of PVI and CFAE ablation in persistent AF leads to acute AF termination in 66% and long-term maintenance of sinus rhythm in 74% of cases. However, long-term outcome was not predictable by acute results of the ablation procedure.

Catecholaminergic Polymorphic Ventricular Tachycardia in Children Analysis of Therapeutic Strategies and Outcomes From an International Multicenter Registry

Background—Catecholaminergic polymorphic ventricular tachycardia is an uncommon, potentially lethal, ion channelopathy. Standard therapies have high failure rates and little is known about treatment in children. Newer options such as flecainide and left cardiac sympathetic denervation are not well validated. We sought to define treatment outcomes in children with catecholaminergic polymorphic ventricular tachycardia. Methods and Results—This is a Pediatric and Congenital Electrophysiology Society multicenter, retrospective cohort study of catecholaminergic polymorphic ventricular tachycardia patients diagnosed before 19 years of age. The cohort included 226 patients, including 170 probands and 56 relatives. Symptomatic presentation was reported in 176 (78%). Symptom onset occurred at 10.8 (interquartile range, 6.8–13.2) years with a delay to diagnosis of 0.5 (0–2.6) years. Syncope (P<0.001), cardiac arrest (P<0.001), and treatment failure (P=0.008) occurred more often in probands. &bgr;-Blockers were prescribed in 205 of 211 patients (97%) on medication, and 25% experienced at least 1 treatment failure event. Implantable cardioverter defibrillators were placed in 121 (54%) and was associated with electrical storm in 22 (18%). Flecainide was used in 24% and left cardiac sympathetic denervation in 8%. Six deaths (3%) occurred during a cumulative follow-up of 788 patient-years. Conclusions—This study demonstrates a malignant phenotype and lengthy delay to diagnosis in catecholaminergic polymorphic ventricular tachycardia. Probands were typically severely affected. &bgr;-Blockers were almost universally initiated; however, treatment failure, noncompliance and subtherapeutic dosing were often reported. Implantable cardioverter defibrillators were common despite numerous device-related complications. Treatment failure was rare in the quarter of patients on flecainide. Left cardiac sympathetic denervation was not uncommon although the indication was variable.

Guidelines for the diagnosis and management of Catecholaminergic Polymorphic Ventricular Tachycardia.

BACKGROUND Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) is an inherited arrhythmia syndrome, characterised by polymorphic ventricular tachycardia induced by adrenergic stress. CPVT can be caused by mutations the cardiac ryanodine receptor gene (RYR2) or mutations in the cardiac calsequestrin gene CASQ2. Structural heart disease is usually absent and the baseline ECG is usually normal. Patients with CPVT often present with exercise- or emotion induced syncope, the first presentation can also be sudden cardiac death. MANAGEMENT Besides removal of triggers treatment with beta blockers is currently a class I indication in clinically diagnosed patients. Beta blockage should be titrated up to an effective level. The addition of flecainide seems to be a promising approach in patients where arrhythmias are not completely suppressed by beta blockers. A cardioverter-defibrillator (ICD) or left cervical sympathetic denervation might be considered under special circumstances. Genetic counselling is recommended and all first degree relatives should be properly evaluated.

Mapping of Intraatrial Reentrant Tachycardias by Remote Magnetic Navigation in Patients with d-Transposition of the Great Arteries After Mustard or Senning Procedure

Introduction: Mapping of intraatrial reentrant tachycardia (IART) still presents a challenge in complex congenital heart disease. The goal of this work was to present our initial experience with remote magnetic navigation (RMN) for mapping of IART in four patients after the atrial switch procedure (Mustard n = 1, Senning n = 3) for d‐transposition of the great arteries.

Predictors of myocardial recovery in pediatric tachycardia-induced cardiomyopathy

BACKGROUND Tachycardia-induced cardiomyopathy (TIC) carries significant risk of morbidity and mortality, although full recovery is possible. Little is known about the myocardial recovery pattern. OBJECTIVE The purpose of this study was to determine the time course and predictors of myocardial recovery in pediatric TIC. METHODS An international multicenter study of pediatric TIC was conducted. Children ≤18 years with incessant tachyarrhythmia, cardiac dysfunction (left ventricular ejection fraction [LVEF] <50%), and left ventricular (LV) dilation (left ventricular end-diastolic dimension [LVEDD] z-score ≥2) were included. Children with congenital heart disease or suspected primary cardiomyopathy were excluded. Primary end-points were time to LV systolic functional recovery (LVEF ≥55%) and normal LV size (LVEDD z-score <2). RESULTS Eighty-one children from 17 centers met inclusion criteria: median age 4.0 years (range 0.0-17.5 years) and baseline LVEF 28% (interquartile range 19-39). The most common arrhythmias were ectopic atrial tachycardia (59%), permanent junctional reciprocating tachycardia (23%), and ventricular tachycardia (7%). Thirteen required extracorporeal membrane oxygenation (n = 11) or ventricular assist device (n = 2) support. Median time to recovery was 51 days for LVEF and 71 days for LVEDD. Two (4%) underwent heart transplantation, and 1 died (1%). Multivariate predictors of LV systolic functional recovery were age (hazard ratio [HR] 0.61, P = .040), standardized tachycardia rate (HR 1.16, P = .015), mechanical circulatory support (HR 2.61, P = .044), and LVEF (HR 1.33 per 10% increase, p=0.005). For normalization of LV size, only baseline LVEDD (HR 0.86, P = .008) was predictive. CONCLUSION Pediatric TIC resolves in a predictable fashion. Factors associated with faster recovery include younger age, higher presenting heart rate, use of mechanical circulatory support, and higher LVEF, whereas only smaller baseline LV size predicts reverse remodeling. This knowledge may be useful for clinical evaluation and follow-up of affected children.

Atresia of the coronary sinus in patients with supraventricular tachycardia

Background: Atresia of the coronary sinus (CS) ostium with retrograde drainage of the cardiac veins via the persistent left superior vena cava (PLSVC) is a rare abnormality and only a few case reports in association with electrophysiological studies have been reported.

Quantification of Periprosthetic Valve Leakage with Multiple Regurgitation Jets by Magnetic Resonance Imaging

A patient with aortic prosthetic valve endocarditis required replacement of the prosthesis, After initial recovery, his left ventricular function gradually declined and echocardiography revealed minor paravalvular leakage and a cavern around the prosthesis. Magnetic resonance imaging was performed, flow-imaging and volume-based measurements showed high-grade paravalvular leakage of 45 and 58% regurgitant fraction, respectively. Intraoperatively, the dehiscence of more than half of the circumference of the aortic prosthesis was seen. This study shows that MRI can accurately assess even periprosthetic valve leakage with multiple regurgitation jets.

COMPASS: A Novel Risk-Adjustment Model for Catheter Ablation in Pediatric and Congenital Heart Disease Patients

BACKGROUND Robust risk-adjustment algorithms are often necessary if data from clinical registries is to be used to compare rates of important clinical outcomes between participating centers. Although such algorithms have been successfully developed for surgical and catheter-based cardiac interventions in children, outcomes of pediatric and congenital catheter ablation have not been modeled with respect to case mix. METHODS A working group was appointed by the Pediatric and Congenital Electrophysiology Society to develop a risk-adjustment algorithm for use in conjunction with a modernized, multicenter registry database. Expert consensus was used to develop relevant outcome measures, an inclusive list of possible predictors, and estimates of associated incremental risk. Historical data from the Pediatric Radiofrequency Ablation Registry was reanalyzed using multivariate regression to create statistical models of ablation outcomes. RESULTS Acute ablation failure and serious adverse event rates were modeled as outcomes. Statistical modeling was performed on 4486 cases performed in 19 centers. For ablation failure rate, a simple model including general category of arrhythmia mechanism and presence of structural congenital heart disease accounted for ∼71% of outcome variance. The model was useful for identification of between-center variability in the historical data set. Although expert consensus predicted the need for a more complex model, predicted univariate effects were similar to those generated by statistical modeling. Serious adverse events were too infrequent to permit statistical association with any predictive variable, but could be compared with the mean rate observed among all centers. CONCLUSION A substantial component of the intercenter variability of acute ablation outcomes in a historical database of pediatric and congenital ablation patients may be accounted for by a simple statistical model, exposing variations in outcome specific to centers. This will be a useful initial model for use a modern registry for pediatric catheter ablation outcomes.

Perspectives in Interventional Electrophysiology in Children and those with Congenital Heart Disease Electrophysiology in Children

Recent developments in paediatric pacing and ablation of arrhythmia substrate have been characterised by adoption and modification of techniques used in adults. Infants, small children and those of all ages with congenital heart disease are a patient group with a higher risk profile needing a special approach. Current success rates for catheter ablation are high and major complication rates are low. Important issues with respect to long-term outcome include questions about coronary injury, long-term effects of radiation exposure and late recurrence. Non-fluoroscopic electro-anatomical mapping systems (3D systems), cryo-ablation and remote navigation are techniques recently improved such that it is possible to potentially reduce fluoroscopy and complications. Pacing in young children and congenital heart disease often warrants an epicardial approach to avoid embolism, venous occlusion and lead failure related to growth. Defibrillator and resynchronisation therapy are increasingly important tools to reduce mortality, although the indications are not as clear as in adult patients without congenital heart disease.