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.

Permanent cardiac pacing in children: choosing the optimal pacing site: a multicenter study.

BACKGROUND: We evaluated the effects of the site of ventricular pacing on left ventricular (LV) synchrony and function in children requiring permanent pacing. METHODS AND RESULTS: One hundred seventy-eight children (aged <18 years) from 21 centers with atrioventricular block and a structurally normal heart undergoing permanent pacing were studied cross-sectionally. Median age at evaluation was 11.2 (interquartile range, 6.3-15.0) years. Median pacing duration was 5.4 (interquartile range, 3.1-8.8) years. Pacing sites were the free wall of the right ventricular (RV) outflow tract (n=8), lateral RV (n=44), RV apex (n=61), RV septum (n=29), LV apex (n=12), LV midlateral wall (n=17), and LV base (n=7). LV synchrony, pump function, and contraction efficiency were significantly affected by pacing site and were superior in children paced at the LV apex/LV midlateral wall. LV dyssynchrony correlated inversely with LV ejection fraction (R=0.80, P=0.031). Pacing from the RV outflow tract/lateral RV predicted significantly decreased LV function (LV ejection fraction <45%; odds ratio, 10.72; confidence interval, 2.07-55.60; P=0.005), whereas LV apex/LV midlateral wall pacing was associated with preserved LV function (LV ejection fraction ≥55%; odds ratio, 8.26; confidence interval, 1.46-47.62; P=0.018). Presence of maternal autoantibodies, gender, age at implantation, duration of pacing, DDD mode, and QRS duration had no significant impact on LV ejection fraction. CONCLUSIONS: The site of ventricular pacing has a major impact on LV mechanical synchrony, efficiency, and pump function in children who require lifelong pacing. Of the sites studied, LV apex/LV midlateral wall pacing has the greatest potential to prevent pacing-induced reduction of cardiac pump function.

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.

Femoral arterial thrombosis after cardiac catheterization in infancy: impact of Doppler ultrasound for diagnosis.

Femoral arterial thrombosis (FAT) is a nonnegligible complication after cardiac catheterization (CC) in infancy. The aim of this study was to evaluate the impact of Doppler ultrasound (US) for diagnostic work-up after catheterization. We compared standard follow-up (FU) without Doppler US by relying on clinical signs of FAT with advanced FU using Doppler US of the femoral vessels. Between January and December 2009, we evaluated the rate of FAT in infants <12 months of age using a multicenter, prospective observational survey. We analysed 171 patients [mean age 4.1 ± 3.3 (SD) months; mean body weight 5.3 ± 1.8 kg] from 6 participating centres. The mean duration of catheter studies was 57.7 ± 38.0 min. The overall rate of FAT based on clinical diagnosis was 4.7% and was comparable in both groups [3.4% undergoing standard FU vs. 7.4% undergoing advanced FU (p = 0.15)]. However, the overall rate of thrombosis as screened by Doppler US was greater at 7.1 %, especially in patients after advanced FU [18.5% advanced vs. standard FU 1.7% (p < 0.01)]. In conclusion, FAT remains a relevant and underestimated complication after catheterization in young infants when relying only on clinical signs of FAT. Therefore, to start effective treatment as soon as possible, we recommend Doppler US to be performed the day after CC.

Lost unilateral capillary perfusion during nonpulsatile pulmonary circulation: successful recovery by oral sildenafil.

Good status of pulmonary perfusion is essential for a successful outcome after the Fontan procedure. Increased pulmonary pressure and vascular resistance, small size of the pulmonary arteries, and significant branch stenoses reflect some of the main problems causing failing Fontan circulation. Here we report a child who underwent a staged Fontan procedure with subsequent subtotal loss of the left-sided pulmonary perfusion, although branch stenosis was successfully treated by stent implantation. Oral sildenafil caused restoration of the capillary vascular bed, improved left-sided lung perfusion, and resulted in significant clinical benefit.

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.

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.

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

[Figure: see text].

Early detection of anomalous origin of left coronary artery from the right pulmonary artery after successful repair of critical coarctation of the aorta.

Left ventricular (LV) function is impaired by increased afterload in neonates with severe coarctation of the aorta, which may result in endocardial fibroelastosis. Repair of the coarctation usually solves the problem, with LV function normalizing after a few weeks. This report describes a patient who underwent successful repair of critical coarctation with normalization of LV function despite signs of endocardial fibroelastosis but with persisting elevation of cardiac troponin T. Cardiac catheterization showed the rare coincidence of anomalous origin of left coronary artery from the right pulmonary artery (ALCAPA) and coronary sinus orifice atresia with left superior vena cava.

Permanent conduction system pacing for congenitally corrected transposition of the great arteries: A Pediatric and Congenital Electrophysiology Society (PACES)/International Society for Adult Congenital Heart Disease (ISACHD) Collaborative Study.

BACKGROUND: Congenitally corrected transposition of the great arteries (CCTGA) is associated with spontaneous atrioventricular block and pacing-induced cardiomyopathy. Conduction system pacing is a potential alternative to conventional cardiac resynchronization therapy (CRT). OBJECTIVE: The purpose of this study was to determine the outcomes of conduction system pacing for CCTGA. METHODS: Retrospective data were collected from 10 international centers. RESULTS: His bundle (HBP) or left bundle branch pacing (LBBP) was attempted in 15 CCTGA patients (median age 23 years; 87% male). Previous surgery had been performed in 8 and chronic ventricular pacing in 7. Conduction system pacing (11 HBP, 2 LBBP 2; nonselective in 10, selective in 3) was acutely successful in 13 (86%) without complication. In 9 cases, electroanatomic mapping was available and identified the distal His bundle and proximal left bundle branches within the morphologic left ventricle below the pulmonary valve separate from the mitral annulus. Median implant HV interval was 42 ms (interquartile range [IQR] 35-48), R wave 6 mV (IQR 5-18), and threshold 0.5 V (IQR 0.5-1.2) at median 0.5 ms. QRSd was unchanged compared to junctional escape rhythm (124 vs 110 ms; P = .17) and decreased significantly compared to baseline ventricular pacing (112 vs 164 ms; P <.01). At a median of 8 months, all patients were alive without significant change in pacing threshold or lead dysfunction. New York Heart Association functional class improved in 5 patients. CONCLUSION: Permanent conduction system pacing is feasible in CCTGA by either HBP or proximal LBBP. Narrow paced QRS and stable lead thresholds were observed at intermediate follow-up. Unique anatomic characteristics may favor this approach over conventional CRT.

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.

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.

Comparison of somatic development and status of conduit after extracardiac Fontan operation in young and older children.

OBJECTIVE: We set out to examine whether the extracardiac Fontan operation (ECFO) in young children is beneficial for the early postoperative course and whether it has a negative impact on the mid-term hemodynamics and growth of the children due to absent growth potential of the prosthetic conduit. Therefore we compared our medium-term experience with ECFO in children under 4 years of age to that in older children regarding the incidence of postoperative complications, somatomotoric development and conduit status. METHODS AND RESULTS: Between 11/95 and 12/02 ECFO was performed in 30 children under 4 years of age and 21 older children aged 4-13 years. There were no deaths in children under 4 years of age and two older children died. No prolonged support (>72 h) of suprarenin was required in small children compared to 4 older children. In twenty-seven children, who underwent postoperative heart catheterization no pulmonary artery or systemic vein distortion occurred. One re-operation and one transcatheter intervention were performed because of the partial conduit stenosis. During the median follow-up of 4.8 years a manifestly accelerated postoperative weight gain was observed in children operated on under 4 years of age, compared to that in older children (up to the 50 vs. 10th percentile, P<0.05). CONCLUSIONS: The ECFO could be performed in young children with low morbidity and mortality rates. In the majority of patients, implanted prosthetic grafts maintain stable form without the development of stenosis. Accelerated somatic development, especially in small children, is to be observed after completion of the Fontan circulation.

[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.

[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.

Impact of the permanent ventricular pacing site on left ventricular function in children: a retrospective multicentre survey.

BACKGROUND: Chronic right ventricular (RV) pacing is associated with deleterious effects on cardiac function. OBJECTIVE: In an observational multicentre study in children with isolated atrioventricular (AV) block receiving chronic ventricular pacing, the importance of the ventricular pacing site on left ventricular (LV) function was investigated. METHODS: Demographics, maternal autoantibody status and echocardiographic measurements on LV end-diastolic and end-systolic dimensions and volumes at age <18 years were retrospectively collected from patients undergoing chronic ventricular pacing (>1 year) for isolated AV block. LV fractional shortening (LVFS) and, if possible LV ejection fraction (LVEF) were calculated. Linear regression analyses were adjusted for patient characteristics. RESULTS: From 27 centres, 297 children were included, in whom pacing was applied at the RV epicardium (RVepi, n = 147), RV endocardium (RVendo, n = 113) or LV epicardium (LVepi, n = 37). LVFS was significantly affected by pacing site (p = 0.001), and not by maternal autoantibody status (p = 0.266). LVFS in LVepi (39 ± 5%) was significantly higher than in RVendo (33 ± 7%, p < 0.001) and RVepi (35 ± 8%, p = 0.001; no significant difference between RV-paced groups, p = 0.275). Subnormal LVFS (LVFS < 28%) was seen in 16/113 (14%) RVendo-paced and 21/147 (14%) RVepi-paced children, while LVFS was normal (LVFS ≥ 28%) in all LVepi-paced children (p = 0.049). These results are supported by the findings for LVEF (n = 122): LVEF was <50% in 17/69 (25%) RVendo- and in 10/35 (29%) RVepi-paced patients, while LVEF was ≥ 50% in 17/18 (94%) LVepi-paced patients. CONCLUSION: In children with isolated AV block, permanent ventricular pacing site is an important determinant of LV function, with LVFS being significantly higher with LV pacing than with RV pacing.

Replacement of the aortic valve after the arterial switch operation.

An 11-month-old infant had undergone a primary arterial switch operation, including the Lecompte maneuver, for correction of discordant ventriculo-arterial connections and closure of an accompanying ventricular septal defect. At discharge, there were no signs of aortic valvar incompetence. Regurgitation across the aortic valve was detected first at the age of 2 years, and then increased progressively, as documented by serial echocardiographic studies. There had been no history of bacterial endocarditis. At the age of 10 years, echocardiography revealed severe aortic valvar incompetence. At operation, the aortic valve had three leaflets, all of which were short, with very restricted movement. Absence of sufficient leaflet tissue precluded a durable valvar reconstruction, so the aortic valve was replaced with a 21 mm mechanical prosthesis. Histological examination of the removed leaflets revealed nodular swelling due to mucous changes of the matrix, as well as fibrous alteration and formation of scar tissue, including areas of fibroblastic and capillary proliferation. There were no signs of calcification or acute inflammatory changes. Improvement of left ventricular function was observed both early postoperatively and later on. Our observation shows that aortic valvar incompetence after an arterial switch operation can be caused by degenerative changes of the neo-aortic leaflets, which prevent plastic reconstruction of the valve, necessitating valvar replacement.

New onset arrhythmias after the extracardiac conduit Fontan operation compared with the intraatrial lateral tunnel procedure: early and midterm results.

BACKGROUND: Arrhythmias are one of the main causes of postoperative morbidity superseding Fontan operations. Comparative data on the incidence of sinus node dysfunction after the extracardiac Fontan operation (ECFO) and the intraatrial lateral tunnel Fontan operation (LTFO) are very limited and controversial. The aim of this study was to evaluate whether ECFO decreases the risk of postoperative arrhythmias compared with LTFO. METHODS: Seventy-four consecutive patients received either an LTFO (n = 29, 5 recordings in 1992 to 9 recordings in 1997) or an ECFO (n = 45, 11 recordings in 1995 to 5 recordings in 2001). The rhythm was documented preoperatively and postoperatively with standard electrocardiogram (ECG) recording and ECG monitoring. During follow-up all patients had 2-8 (median 3) standard ECG recordings per year. Additionally 45 patients (65%) had a Holter ECG at least once a year. RESULTS: Median follow-up post-ECFO was 4.4 years (1.6-7.2) and post-LTFO it was 7.9 years (5.4-11.1). There were 5 early deaths (3 LTFO, 2 ECFO) and 1 late death (LTFO) (total mortality 8%). Sinus rhythm persisted in 37 ECFO patients (86%) as compared with 13 LTFO patients (50%) (p < 0.001). The incidence of new onset supraventricular tachyarrhythmias (SVTs) post-ECFO compared with LTFO was lower: 5 patients (11%) versus 11 patients (38%) early postoperatively (p < 0.001) and none versus 7 patients (27%) during follow-up (p < 0.001), respectively. Early postoperatively 10 LTFO patients (34%) and another 3 patients during follow-up required permanent pacemaker implantation due to bradyarrhythmias, but none of the ECFO patients required this. CONCLUSIONS: Our data suggest that ECFO decreases the incidence of postoperative new onset arrhythmias during early and midterm follow-up compared with LTFO.