Williams–Beuren syndrome (WBS) is a congenital multisystem disorder affecting the cardiovascular, central nervous, and musculoskeletal systems. Cardiovascular abnormalities, which consist principally of vascular stenoses, occur in approximately 80% of people with WBS and are the predominant cause of early morbidity and mortality. Supravalvar aortic stenosis and peripheral pulmonary artery stenosis are the most common stenotic lesions in WBS, though other stenoses often occur, including stenoses of the coronary arteries. Approximately one‐third of people with WBS undergo cardiovascular interventions. The risk of sudden cardiac death is markedly higher than the general population, with most events occurring in the periprocedural period. Because of the rarity of WBS and the often‐complex nature of the cardiovascular abnormalities, most physicians, including cardiologists, have limited experience in caring for patients with WBS. Further, heretofore, clinical cardiovascular management guidelines based on international expert consensus have not been available. This state‐of‐the‐art review provides a comprehensive synopsis of the cardiovascular abnormalities in WBS and presents clinical management guidelines based on the authors' expert consensus.

HomeCirculation: Arrhythmia and ElectrophysiologyAhead of PrintPulsed Field Ablation of Atrial Fibrillation and Atrial Tachycardia in Adult Patients With Congenital Heart Disease No AccessLetterRequest AccessAboutView PDFSections ToolsAdd to favoritesDownload citationsTrack citationsPermissions ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toNo AccessLetterRequest AccessPulsed Field Ablation of Atrial Fibrillation and Atrial Tachycardia in Adult Patients With Congenital Heart Disease Ulrich Krause, Leonard Bergau, Markus Zabel, Heike E. Schneider, Matthias J. Müller and Thomas Paul Ulrich KrauseUlrich Krause Correspondence to: Ulrich Krause, MD, Department of Pediatric Cardiology and Intensive Care Medicine, University Medical Center, Georg-August-University Göttingen, Robert-Koch-Str. 40, 37099 Göttingen, Germany. Email E-mail Address: [email protected] https://orcid.org/0000-0003-3673-165X Department of Pediatric Cardiology, Intensive Care Medicine and Neonatology, University Medical Center, Georg-August-University Göttingen, Germany. (U.K., H.E.S., M.J.M., T.P.) , Leonard BergauLeonard Bergau https://orcid.org/0000-0001-7025-9315 Department of Cardiology and Pneumology, Heart Center, University Medical Center, Georg-August-University Göttingen, Germany. (L.B., M.Z.) , Markus ZabelMarkus Zabel https://orcid.org/0000-0003-0895-4602 Department of Cardiology and Pneumology, Heart Center, University Medical Center, Georg-August-University Göttingen, Germany. (L.B., M.Z.) , Heike E. SchneiderHeike E. Schneider https://orcid.org/0000-0001-5946-4342 Department of Pediatric Cardiology, Intensive Care Medicine and Neonatology, University Medical Center, Georg-August-University Göttingen, Germany. (U.K., H.E.S., M.J.M., T.P.) , Matthias J. MüllerMatthias J. Müller https://orcid.org/0000-0001-9511-1093 Department of Pediatric Cardiology, Intensive Care Medicine and Neonatology, University Medical Center, Georg-August-University Göttingen, Germany. (U.K., H.E.S., M.J.M., T.P.) and Thomas PaulThomas Paul https://orcid.org/0000-0003-1895-6345 Department of Pediatric Cardiology, Intensive Care Medicine and Neonatology, University Medical Center, Georg-August-University Göttingen, Germany. (U.K., H.E.S., M.J.M., T.P.) Originally published23 Apr 2024https://doi.org/10.1161/CIRCEP.123.012698Circulation: Arrhythmia and Electrophysiology. 2024;0:e012698FootnotesFor Sources of Funding and Disclosures, see page XXX.Correspondence to: Ulrich Krause, MD, Department of Pediatric Cardiology and Intensive Care Medicine, University Medical Center, Georg-August-University Göttingen, Robert-Koch-Str. 40, 37099 Göttingen, Germany. Email ukrause1@gwdg.de eLetters(0)eLetters should relate to an article recently published in the journal and are not a forum for providing unpublished data. Comments are reviewed for appropriate use of tone and language. Comments are not peer-reviewed. Acceptable comments are posted to the journal website only. Comments are not published in an issue and are not indexed in PubMed. Comments should be no longer than 500 words and will only be posted online. References are limited to 10. Authors of the article cited in the comment will be invited to reply, as appropriate.Comments and feedback on AHA/ASA Scientific Statements and Guidelines should be directed to the AHA/ASA Manuscript Oversight Committee via its Correspondence page.Sign In to Submit a Response to This Article Previous Back to top Next FiguresReferencesRelatedDetails Advertisement Article InformationMetrics © 2024 American Heart Association, Inc.https://doi.org/10.1161/CIRCEP.123.012698PMID: 38651354 Originally publishedApril 23, 2024 Keywordsatrial fibrillationcoronary vesselsheart defects, congenitalpulmonary veinstachycardiaPDF download Advertisement SubjectsAtrial FibrillationCongenital Heart Disease

In patients with AV-block (AVB), His bundle pacing (HBP) allows physiological ventricular depolarisation and may prevent pacemaker-induced cardiomyopathy. Selective and non-selective HBP is a feasible procedure in pediatric patients with and without congenital heart disease (CHD).

Patients with dextro transposition of the great arteries (d-TGA) after atrial switch procedure are at risk to develop heart failure and arrhythmias during long-term follow-up. The present study aims to add knowledge on the fate of subjects after Mustard procedure during long-term follow-up into adulthood. A single center, retrospective chart review analysis was conducted. All subjects who had Mustard-type atrial switch procedure between 1969 and 1994 at our institution were included. A total of 92 subjects were included. Early postoperative death was reported in 2 subjects. Long-term follow-up was available in 49 survivors. Of those, 6 individuals died during further follow-up. Sudden cardiac death was the most prevalent cause for fatal outcome. Mortality during long-term follow-up was associated with the presence of additional cardiovascular malformations (complex d-TGA). Sinus node dysfunction was observed in 65% of the patients and atrial tachyarrhythmias were common in adult survivors (63%). Implantation of a pacemaker or a cardioverter defibrillator was required in 31% and 45% of those surviving into adulthood. Complications were frequently observed during follow-up after either pacemaker or cardioverter defibrillator implantation (43%) with lead failure being the most frequent complication. The aging population of patients after Mustard procedure is facing challenging problems mainly resulting from a failing systemic right ventricle, presence of associated cardiac malformations and the presence of atrial baffles associated with relevant atrial scars. Age, associated cardiac malformations, and atrial tachyarrhythmias seem to play a major role in determining the fate of patients with d-TGA after atrial switch procedures.

BACKGROUND Accessory atrioventricular pathways (APs) are the most common tachycardia substrate for supraventricular tachy-cardia (SVT) in the young. Endocardial catheter ablation of AP may be unsuccessful in up to 5% of patients because of a coronary sinus location.OBJECTIVE The purpose of this study was to obtain data on abla-tion of accessory pathways within the coronary venous system (CVS) in the young.METHODS Analysis of feasibility, outcome, and safety in patients <18 years with coronary sinus accessory pathways (CS-APs) and catheter ablation via CVS in a tertiary pediatric electrophysiological referral center (May 2003 to December 2021) was performed. The control group adjusted for age, weight, and pathway location was established from patients of the prospective European Multicenter Pediatric Ablation Registry who all had undergone endocardial AP ablation.RESULTS Twenty-four individuals underwent mapping and in-tended AP ablation within the CVS (age 2.7-17.3 years; body weight 15.0-72.0 kg). Because of proximity to the coronary artery, ablation was withheld in 2 of the patients. Overall procedural success was achieved in 20 of 22 study patients (90.9%) and in 46 of 48 controls (95.8%). Coronary artery injury after radiofrequency ablation was noted in 2 of 22 study patients (9%) and in 1 of 48 controls (2%). In CVS patients, repeat SVT occurred in 5 of 22 patients (23%) during median follow-up of 8.5 years, and 4 of the 5 under-went reablation, resulting in 94.4% overall success. Controls were free from SVT during follow-up of 12 months as defined by the reg-istry protocol.CONCLUSION Success of CS-AP ablation in the young was compa-rable to that of endocardial AP ablation. Substantial risk of coronary artery injury should be considered when CS-AP ablation is performed in the young.

Background Prevalence of atrial fibrillation (AF) is increasing in adult patients with congenital heart disease (CHD). Experience using the cryoballoon to achieve pulmonary vein isolation (PVI) in adult CHD patients is limited. The aim of the present study was to assess the value of PVI by cryoballoon in adult CHD patients and to evaluate the significance of additional radiofrequency (RF) ablation of atrial tachycardia (AT). Patients and methods Prospective data analysis; all patients with CHD and AF and PVI using the cryoballoon from January 2017 through November 2021 were included. Results Nineteen patients with various types of CHD were included. Median age was 58 (IQR 47-63) years. A total of 12/19 (63%) patients had had RF ablation of right atrial AT before. Median procedure duration was 225 (IQR 196-261) min. Median fluoroscopy time was 12.3 (IQR 5.2-19.5) min and median freeze time was 32 (IQR 28-36.3) min. Procedural success was achieved in all patients. Additional RF catheter ablation of intraatrial reentrant tachycardia within the left atrium was performed in 3/19 (16%) subjects and within the right atrium in 6/19 (32%) patients. Median follow-up was 26 (IQR 9-49) months. Excluding a 90-day blanking period, recurrence of AF was observed in 6/19 subjects (32%). After one redo procedure deploying RF energy only, 84% of all patients remained free from recurrence. Phrenic nerve palsy was observed in 1 subject. Conclusion Results after PVI using the cryoballoon plus additional RF ablation of AT were promising (84% success including one redo procedure). Success of AF ablation was unsatisfactory in all patients who had no additional AT ablation. Ablation of any AT in these patients should therefore be considered in addition to PVI.

INTRODUCTION:Catheter contact is a key determinant for lesion size in radiofrequency catheter ablation (RFA). Monitoring of contact force (CF) during RFA has been shown to improve efficacy of RFA in experimental settings as well as in adult patients. Coronary artery narrowing after RFA has been described in experimental settings as well as in children and adults and may be dependent from catheter contact. The value of CF monitoring concerning these issues has not been systematically studied yet. Value of high versus low CF during RFA in piglets was studied to assess lesion size and potential coronary artery involvement mimicking RFA in small children.METHODS:RFA with continuous CF monitoring was performed in 24 piglets (median weight 18.5 kg) using a 7 F TactiCath Quartz radiofrequency (RF) ablation catheter (Abbott). A total of 7 lesions were induced in each animal applying low (10-20 g) or high (40-60 g) CF. RF energy was delivered with a target temperature of 65°C at 30 W for 30 s. Coronary angiography was performed prior and immediately after RF application. Animals were assigned to repeat coronary angiography followed by heart removal after 48 h (n = 12) or 6 months (n = 12). Lesions with surrounding myocardium were excised, fixated, and stained. Lesion volumes were measured by microscopic planimetry.RESULTS:A total of 148 RF lesions were identified in the explanted hearts. Only in the subset of lesions at the AV annulus 6 month after ablation, lesion size and number of lesions exhibiting transmural extension were higher in the high CF group compared to low CF. In all other locations CF had no impact on lesion size and mural extension after 48 h as well as after 6 months. Additional parameters such as lesion size index and force time integral were also not related to lesion size. Coronary artery damage was present in two animals after 48 h and in one after 6 months and was not related to CF.CONCLUSION:In our experimental setting, lesion size in piglets was not related to catheter CF. Transmural extension of the RF lesions involving the layers of the coronary arteries was frequently noted irrespective of CF. Coronary artery narrowing was present in 3/24 animals and was not related to CF. In infants and toddlers, low CF (10-20 g) may be of adequate effect. Impact of CF monitoring during conventional RF ablation in children requires further investigation.

Objectives. Aim of this study was to evaluate the long-term course of patients with congenital heart disease (CHD) requiring repeat ablation procedures (RAP) of atrial tachycardias (AT). Background. Reports on RAP of AT in patients with CHD are sparse.Patients and Methods. All 144 patients with CHD who had undergone ablation of AT at our center between January 2003 and October 2018 were enrolled. Patients were classified according to complexity of CHD: complex CHD (cCHD), moderate CHD (mCHD) and simple CHD (sCHD). Results. A total of 101 RAP were performed in 64 patients. One RAP was performed in n=40, two in n=13, three in n=10 and 5 in n=1. Acute success rate was 82% (83/101) and was not associated with complexity of CHD (p=1.0). Mean number of procedures was lower in patients with sCHD than in patients with mCHD and cCHD (sCHD 1.3{plus minus}0.6, mCHD 1.8{plus minus}1.0 and cCHD 1.8{plus minus}1.1, p=0.04). RAP were most frequent in patients after Fontan palliation or atrial switch procedure (2.0{plus minus}1.1 (n=41) vs. 1.6{plus minus}0.9 all others, p=0.016) and in patients with multiple unstable AT´s (2.5{plus minus}1.1 (n=11) vs. 1.7{plus minus}1.0, p=0.008). Major complications occurred in 4/101 procedures. Complete follow-up was available in 125 patients. Since last RAP 73% of the patients were in sinus/atrial rhythm and 34/125 patients (27%) with AT recurrence did not require re-ablation with mean follow-up of 52{plus minus}40 months. Conclusions. Recurrences after ablation of AT in CHD patients were frequent. After RAP promising long-term results could be achieved. Data encourage repetitive ablation procedures in this patient population.

Objectives: Atrioventricular (AV) canal defects are rare but complex congenital diseases characterized by the presence of abnormal communications between the atria and ventricles along with malformations of the AV valves. These defects lead to pulmonary overload resulting in pulmonary hypertension and heart failure. Down syndrome is often associated with AV-canal defects. The aim of the study was the analysis of outcomes after correction of AV-canal defects.

Dieser Übersichtsbeitrag beschreibt die zunehmende Bedeutung von Herzrhythmusstörungen auf die Langzeitprognose von Erwachsenen mit einem angeborenen Herzfehler (EMAH). Tachykarde Herzrhythmusstörungen sind die häufigste Ursache für eine notfallmäßige Aufnahme von EMAH in eine Klinik. Mit zunehmendem Lebensalter kommt es zum deutlich erhöhten Auftreten von supraventrikulären und ventrikulären Tachyarrhythmien bei diesen Patienten. Generell ist festzustellen, dass bei den Betroffenen ein im Vergleich zur Normalbevölkerung um den Faktor 2 erhöhtes Risiko für schwerwiegende Komplikationen wie einen Schlaganfall und eine Herzinsuffizienz sowie für die Notwendigkeit operativer oder katheterinterventioneller Eingriffe besteht. Bei ventrikulären Tachyarrhythmien besteht das Risiko des plötzlichen Herztods.

Die normale fetale Herzfrequenz beträgt zwischen 110 und 180 Schlägen/min. Intrauterine Arrhythmien sind nicht selten. Die fetale Echokardiographie erlaubt in den meisten Fällen eine exakte Diagnose. Die häufigste Ursache von fetalen Arrhythmien sind Vorhofextrasystolen, welche in der Regel hämodynamisch gut toleriert werden, keine Therapie benötigen und eine sehr gute Prognose aufweisen. Intrauterine Bradykardien (Herzfrequenz < 110 Schläge/min) basieren auf einer Sinusknotendysfunktion, einem kompletten AV-Block oder einem blockierten atrialen Bigeminus. Der isolierte, antikörpervermittelte AV-Block III° ist in der Regel irreversibel, der Nutzen einer medikamentösen intrauterinen Therapie bei AV-Block III° zur Verbesserung der Prognose nicht gut belegt. Die meisten Kinder benötigen nach der Geburt die Implantation eines antibradykarden Herzschrittmachersystems. Intrauterine Tachykardien (Herzfrequenz > 180 Schläge/min) sind meist durch supraventrikuläre Tachykardien und Vorhofflattern verursacht. Diese können zuverlässig diagnostiziert und effektiv medikamentös behandelt werden; eine kardiale Dekompensation mit Ausbildung eins Hydrops fetalis ist sehr selten.

Transseptal puncture (TSP) is a standard procedure to obtain access to the left heart. However, data on TSP in infants and children particularly with congenital heart defects (CHD) is sparse. Safety and efficacy of TSP in infants and children < 18 years with normal cardiac anatomy and with CHD were assessed. 327 TSP were performed in a total of 300 individuals < 18 years from 10/2002 to 09/2018 in our tertiary pediatric referral center. Median age at TSP was 11.9 years (IQR 7.8–15; range: first day of life to 17.9 years). 13 subjects were < 1 year. Median body weight was 43.8 kg (IQR 26.9–60; range: 1.8–121 kg). CHD was present in 28/327 (8.6%) procedures. TSP could be successfully performed in 323/327 (98.8%) procedures and was abandoned in 4 procedures due to imminent or incurred complications. Major complications occurred in 4 patients. 3 of these 4 subjects were ≤ 1 year of age and required TSP for enlargement of a restrictive atrial septal defect in complex CHD. Two of these babies deceased within 48 h after TSP attempt. The third baby needed urgent surgery in the cath lab. Pericardial effusion requiring drainage was noted in the forth patient (> 1 year) who was discharged well later. Minor complications emerged in 5 patients. The youngest of these individuals (0.3 years, 5.8 kg) developed small pericardial effusion after anterograde ballon valvuloplasty for critical aortic stenosis. The remaining 4/5 patients developed small pericardial effusion after ablation of a left-sided accessory atrioventricular pathway (6.1–12.2 years, 15.6–34.0 kg). TSP for access to the left heart was safe and effective in children and adolescents > 1 year of age. However, TSP was a high-risk procedure in small infants with a restrictive interatrial septum with need for enlargement of interatrial communication.

The normal fetal heart rate ranges between 110 und 180 beats per minute (bpm). Intrauterine arrhythmias are not an uncommon finding. Fetal echocardiography (ECG) allows for correct diagnosis of the arrhythmia, which is prerequisite for decision making and treatment. Most fetal rhythm disturbances are the result of premature atrial contractions and are of little clinical significance. Intrauterine bradycardias (heart rate < 110 bpm) result from sinus node dysfunction, complete AV block and nonconducted atrial bigeminy. Isolated complete heart block related to maternal anti-SSA/Ro or SSB/La auto-antibodies is irreversible in almost all fetuses. Anti-inflammatory therapy and chronotropic medication may improve outcome. Newborn babies often require pacemaker implantation to augment cardiac output. Intrauterine tachycardias (heart rate > 180 bpm) are most commonly related to supraventricular tachycardia and atrial flutter. Specific antiarrhythmic medication is available to stop the arrhythmia and to prevent hemodynamic deterioration.

This review article highlights the increasing importance of cardiac arrhythmias for the long-term prognosis of adults with congenital heart diseases (ACHD). Tachyarrhythmias are the most frequent reason for an emergency hospital admission of young ACHD. The lifetime incidence of supraventricular and ventricular tachyarrhythmias in these patients increases steadily with age. In general, it can be stated that these patients have an increased risk by a factor of 2 compared to the normal population of severe complications, such as stroke, heart failure and the necessity for surgical or catheter interventions. In cases of ventricular tachyarrhythmia there is the risk of sudden cardiac death.

Catheter-interventional treatment is a growing field in pediatric cardiology and cardiology, replacing an increasing number of operations. This article provides an overview of the general practice of hygienic measures and antimicrobial prophylaxis in the cardiac catheterization laboratory to prevent post-procedural infection, particularly if foreign material is utilized.

We used a new grid-style multi-electrode mapping catheter (Advisor™ HD Grid, Abbott) and investigated its use for high density mapping of atrial tachycardias in adult patients with congenital heart disease. All patients with congenital heart disease who had mapping of atrial tachycardias using the new grid-style catheter between March 2018 and April 2019 were included. A total of 24 adult patients had high density mapping of atrial tachycardias using the grid-style multi-electrode catheter. Mean procedure duration was 207 ± 72 min., mean fluoroscopy time was 7.1 ± 7.9 min. In patients with right atrial substrates, fluoroscopy time was shorter compared to biatrial or left atrial substrates (0.9 ± 2.2 min for right atrial substrates, n = 19 vs. 6.3 ± 8.3 min for left atrial substrates, n = 2 and 7.5 ± 4.3 min for biatrial substrates, n = 3, p = 0.01). A mean number of 14.814 ± 10.140 endocardial points were collected and 2.319 ± 1244 points were finally used to characterize the tachycardia. Procedural success was achieved in 21/24 (88%) subjects and partial success in 2/24 (8%) patients. Recurrence rate was low (12.5%). In one patient, radiofrequency ablation within the cavotricuspid isthmus resulted in occlusion of a branch of the right coronary artery. No complications related to the use of the mapping catheter itself occurred. High density mapping of AT using the grid-style catheter showed promising results with respect to procedural and midterm outcome and fluoroscopy time. Using the grid-style catheter might offer advantages compared to other multi-electrode catheters used for high density mapping of AT in patients with CHD.