Device therapy to prevent sudden death in patients with structural heart disease.

The advent of the implantable cardioverter defibrillator has provided clinicians with a potential tool to prevent sudden arrhythmic death. When considering patients with structural heart disease, long-term follow-up data have suggested that this is indeed an important cause of late mortality. It is essential therefore to undertake follow-up studies to identify high risk individuals or disease categories that are associated with sudden cardiac death (SCD), and to elucidate the specific risk factors that may be associated with this complication. We provide a brief update on the current state of knowledge in this challenging and rapidly developing field.

Risk stratification in young patients with channelopathies.

Identifying the young patient at risk of malignant arrhythmias and sudden cardiac death remains a challenge. It is increasingly recognised that sudden death, syncope and aborted cardiac arrest at a young age in patients with a structurally normal heart may be the result of various ion channel disorders - the channelopathies. The approach to risk stratification involves a combination of the clinical presentation, taken in conjunction with the family history, genetic testing, invasive electrophysiological studies or other provocative tests where appropriate and feasible. A logical approach to risk stratification in some of the commoner channelopathies seen in paediatric practice is presented.

[Smith-Lemli-Opitz syndrome--case report, diagnostics and therapeutic options]. / Das Smith-Lemli-Opitz Syndrom--Fallbericht, Diagnostik, therapeutische Optionen.

Smith-Lemli-Opitz syndrome (SLOS) is an autosomal-recessive disease characterised by the combination of (foetal) growth retardation, mental retardation and a typical malformation pattern. In particular, the combination of cardiovascular defects, Y-shaped syndactyly of the 2 (nd) and 3 (rd) toes and a distinctive craniofacial appearance, often including a cleft palate, are characteristic of SLOS. The disease is caused by a defect in cholesterol synthesis resulting in a reduced or absent activity of the enzyme 7-dehydrocholesterol reductase (DHCR7). As a consequence, a lack of cholesterol and an increase of toxic cholesterol precursors are observed in the majority of patients. We report on a female patient who was born at 37 weeks of gestation and was both small and light for gestational age who displayed typical signs of SLOS. After the diagnosis had been confirmed, a therapeutic approach with oral substitution of cholesterol and the administration of simvastatin was initiated. In spite of this strategy, the patient died at the age of 12 weeks from the disease. Based on the case presented, we review and discuss current diagnostic and therapeutic options for patients with SLOS.

Catheter ablation in ASymptomatic PEDiatric patients with ventricular preexcitation: results from the multicenter "CASPED" study.

BACKGROUND: As there are limited data about the clinical practice of catheter ablation in asymptomatic children and adolescents with ventricular preexcitation on ECG, we performed the multicenter "CASPED" (Catheter ablation in ASymptomatic PEDiatric patients with Ventricular Preexcitation) study. METHODS AND RESULTS: In 182 consecutive children and adolescents aged between 8 and 18 years (mean age 12.9 ± 2.6 years; 65% male) with asymptomatic ventricular preexcitation, a total of 196 accessory pathways (APs) were targeted. APs were right sided (62%) or left sided (38%). The most common right-sided AP location was the posteroseptal region (38%). Ablation was performed using radiofrequency (RF) energy (93%), cryoablation (4%) or both (3%). Mean procedure time was 137.6 ± 62.0 min with a mean fluoroscopy time of 15.6 ± 13.8 min. A 3D mapping or catheter localization system was used in 32% of patients. Catheter ablation was acutely successful in 166/182 patients (91.2%). Mortality was 0% and there were no major periprocedural complications. AP recurrence was observed in 14/166 patients (8.4%) during a mean follow-up time of 19.7 ± 8.5 months. A second ablation attempt was performed in 20 patients and was successful in 16/20 patients (80%). Overall, long-term success rate was 92.3%. CONCLUSION: In this retrospective multicenter study, the outcome of catheter ablation for asymptomatic preexcitation in children and adolescents irrespective of antegrade AP conduction properties is summarized. The complication rate was low and success rate was high, the latter mainly depending on pathway location. The promising results of the study may have future impact on the ongoing risk-benefit discussion regarding catheter ablation in the setting of asymptomatic preexcitation in children and adolescents.

Stem cells in pediatric heart failure.

Pediatric heart failure could be a target for regenerative therapy. Stem cell-based therapy has the potential to provide functional cardiomyocytes. Whereas adult stem cells have shown no or only minimal therapeutic benefit in adults with no evidence of transdifferentiation, embryonic stem cells can differentiate to any cell type, including cardiomyocytes. However, ethical concerns and immunological problems are associated with embryonic stem cells derived from the inner cell mass of blastocysts. Recently, somatic cells could be reprogrammed to a pluripotent state (i.e. induced pluripotent stem cells) with the help of transcription factors. This technique removes ethical and probably also immunological concerns. Nevertheless extensive experimental research will be necessary before cell replacement strategies become clinically applicable. Because the underlying pathophysiology differs significantly with age, caution is warranted extrapolating data obtained in experimental models of cardiac ischemia and clinical studies in adults to the pediatric population. Pediatric heart failure has a good prognosis if causal therapy is possible. However, some forms of congenital heart disease and especially dilated cardiomyopathy still have limited therapeutic options. Almost half of children with symptomatic cardiomyopathy receive a transplant or die within two years. The authors will review the relevant stem cell sources for cell-based treatments. And, given the differences of the underlying diseases between adult and pediatric patients with heart failure, it is contemplated which condition of pediatric patients with heart failure is most likely to benefit and which cell type would be appropriate.

Paediatric electrophysiologic studies: how and what with?

Catheter ablation of arrhythmias in children has become standard practice virtually worldwide. Successful and safe ablation has been made possible by a combination of factors. These include increased operator experience, a better understanding of the natural history of a wide variety of arrhythmias, advances in technology such as smaller catheters, the routine use of various three-dimensional mapping systems, and the development of alternative energy sources. It is also not uncommon to perform multiple catheter intervention procedures (ablation +/- intravascular stent implantation +/- device closure of residual shunts +/- elective pacemaker or device implantation) during a single session. It is important to bear in mind that arrhythmia recurrence is commoner in children in general, and that this is particularly the case with postoperative (scar-related arrhythmias). Despite acute success, long-term follow-up is mandated for this subgroup of patients.

[Sensorineural loss of hearing in lower registers as the main symptom of Lyme disease]. / Symmetrische Tieftonschwerhörigkeit als Hauptsymptom einer Neuroborreliose.

In a 9-year-old boy with sudden sensorineural loss of hearing in the lower registers in both ears, serology showed elevated levels of antibodies against Borrelia burgdorferi and examination of the CSF revealed a positive antibody index against Borrelia burgdorferi. The boy was treated with antibiotics for 2 weeks. Audiometry performed 4 weeks after treatment was completely normal. Inner ear involvement in Lyme disease has often been discussed. Treating these patients with antibiotics may lead to an improvement in some.

Intracardiac rhabdomyomas producing symptoms in infancy: the role of radiofrequency catheter ablation.

BACKGROUND: Cardiac rhabdomyomas, although benign, may produce symptoms related to arrhythmia or mechanical obstruction. Surgical excision is the therapy of choice for symptomatic rhabdomyomas in infancy. PATIENTS AND METHODS: Two infants with intracardiac rhabdomyomas producing symptoms underwent radiofrequency catheter ablation of the tumour. In patient 1 the diagnosis of multiple rhabdomyomas associated with recurrent supraventricular tachyarrhythmias and foetal hydrops was made in utero. After birth, several antiarrhythmic agents were administered, without successful suppression of the tachyarrhythmia. At seven months of age, the infant had one large residual tumour on the left atrial aspect of the anterior mitral valve leaflet with associated pre-excitation and re-entrant supraventricular tachyarrhythmia suggestive of a left-sided pathway. Catheter ablation of the accessory pathway was performed via a retrograde femoral arterial approach, targeting the earliest site of ventricular activation. Patient 2 presented as a neonate with multiple rhabdomyomas, one of which, measuring 15 mm × 15 mm, was producing severe mitral valve inflow obstruction resulting in symptoms of heart failure due to a large left-to-right shunt at atrial level and persistent pulmonary hypertension. Via the femoral vein, a 5F ablation catheter was advanced across the atrial septum, and the tumour directly ablated. RESULTS: Echocardiography performed 24 hours later demonstrated alteration in tumour morphology, with the development of a large central echolucent area, followed by progressive tumour shrinkage in both infants. Patient 1 was discharged at 24 hours, and patient 2 at seven days post-ablation, without symptoms. Follow-up at four weeks confirmed further tumour shrinkage. CONCLUSION: Transcatheter tumour ablation may be beneficial in selected infants and children.

Radiofrequency catheter septal ablation for hypertrophic obstructive cardiomyopathy in children.

BACKGROUND: The definitive therapeutic options for symptomatic obstructive cardiomyopathy in childhood are restricted. At present, extensive surgical myectomy is the only procedure that is of proven benefit. PATIENTS AND METHODS: Three patients, aged 5, 11 and 17 years, respectively, with progressive hypertrophic obstructive cardiomyopathy and increasing symptoms were considered for radiofrequency catheter septal ablation. The peak Doppler gradient recorded on several occasions ranged between 50 to 90mmHg. Via a femoral arterial approach, the His bundle was initially plotted and marked using the LocaLisa navigation system. Subsequently, using a cooled tip catheter a series of lesions were placed in the hypertrophied septum, taking care to stay away from the His bundle. A total of 17, 50 and 45 lesions were applied in the three patients. In one case, the procedure was complicated by two episodes of ventricular fibrillation requiring DC cardioversion but without any neurological sequelae. RESULTS: The preablation peak-to-peak gradient between left ventricle and aorta was 50 mmHg, 60 mmHg and 60 mmHg, respectively, and remained unchanged immediately after the procedure. All patients were discharged from hospital 48 hours later. Serial measurement of serum troponin T and CK-MB isoenzyme confirmed significant myocardial necrosis. Follow-up echocardiography both at seven days and at six weeks postablation confirmed a beneficial haemodynamic result, with reduction of left ventricular outflow obstruction and relief of symptoms. CONCLUSION: In young children, in whom alcohol-induced septal ablation is not an option, radiofrequency catheter ablation offers an alternative to surgery, with the benefits of repeatability and a lower risk of procedure-related permanent AV block.

[Electrical heart diseases--therapy during childhood and adolescence]. / Elektrische Herzerkrankungen--Behandlung im Kindes- und Jugendalter.

With increasing experience, radiofrequency catheter ablation of tachyarrhythmia substrates has become first choice therapy for children >4 years of age with recurrent tachyarrhythmia. In younger patients, the risks associated with the procedure (typically procedure-related AV block or possible coronary artery damage) have to be weighed against the natural history of the tachyarrhythmia substrate, and the degree of control achieved with pharmacologic agents. Ablation for postoperative arrhythmias is more complicated, and associated with lower success rates and a higher rate of recurrence (of the same or a new tachyarrhythmia) despite acute procedural success. In this setting, catheter ablation has to be considered in conjunction with further surgery or the use of a defibrillator as a backup device to prevent arrhythmia-related sudden death. Also in inherited arrhythmias as in long QT syndrome and Brugada syndrome, implantable defibrillators have to be considered as a possible therapeutic option for patients with a higher risk for sudden cardiac death, irrespective of age.

Risk factors of hepatocellular carcinoma in Germany: hepatitis B or liver cirrhosis?

In a retrospective study, 42 out of 44 cases of hepatocellular carcinoma were investigated immunohistochemically for chronic hepatitis B infection. Surface antigen was found in the liver tissue of only 4 of these cases. In 41 of the patients, mildly to moderately active cirrhosis of the liver was found to be underlying the carcinoma. The age distribution and case histories showed that hepatocellular carcinoma often developed from low-complication cirrhosis of long standing and of various etiologies, and must thus be considered a late complication of cirrhosis.

Transhepatic approach for catheter ablation of accessory pathway in a child with complex congenital heart disease.

We report on a 22-month-old boy with drug-resistant atrioventricular reentrant tachycardia and complex structural heart disease consisting of right atrial isomerism, mirror image orientation of the intrathoracic veins, hemi-azygos continuation to the left superior vena cava, separate drainage of the hepatic veins into the left-sided atrium, congenitally corrected transposition, pulmonary atresia, and atrial and ventricular septal defects. Access to the heart for radiofrequency (RF) ablation was obtained by percutaneous puncture of a hepatic vein, the left internal jugular vein, and femoral artery. The accessory pathway was localised to the free wall of the left-sided AV groove and successfully ablated. There were no procedure-related complications. RF ablation of an accessory pathway is feasible in young children with complex structural heart disease and abnormal systemic venous return. In such patients access to the heart must be planned with knowledge of the anatomy and judicious use of the hepatic venous approach.

[Cryoenergy for the ablation of arrhythmogenic paraseptal substrates in children and adolescents with heart rhythm disorders]. / Kryoenergie zur Ablation arrhythmogener paraseptaler Substrate bei Herzrhythmusstörungen im Kindes- und Jugendalter.

BACKGROUND AND OBJECTIVE: Cryoablation is safe for the ablation of substrates in proximity to the AV node, because the initial lesion is reversible. We report our results of cryoablation in a transregional center for ablation in children and adolescents. PATIENTS AND METHODS: Data on 39 children and adolescents (4 - 18 years of age) who had been treated with cryo energy were analyzed retrospectively. The diagnosis was AV nodal reentry tachycardia (AVNRT; n = 30), para-Hisian accessory pathway (AP; n = 6) and congenital junctional ectopic tachycardia (JET; n = 4). In addition to non-inducibility, the targeted endpoint for AP-ablation was a missing or decremental concentric retrograde conduction, for ablation of AVNRT the endpoint was no slow pathway, no AH jumps and no echo-beats. The median follow-up was 3 years (270 - 1919 days). RESULTS: The targeted endpoint was reached in 35/39 patients (90 %), in four patients (10 %) RF energy had to be applied. A recurrence occurred in 7/35 (20 %) successfully treated patients. Two patients had a pre-excitation again after AP ablation, but no symptoms. Thus, 28/35 patients (80 %) remained asymptomatic after cryoablation, and 26/35 (74 %) are definitively cured, regarding all follow-up data. The subgroup of AVNRT patients does not differ from the entire group. There was no AV block in the cryoablation group. CONCLUSIONS: Cryoablation is very safe and effective for the definitive treatment of arrhythmias in children and adolescents. The price for the high safety is a reduced efficacy and a higher recurrence rate.

Balloon valvuloplasty in the treatment of congenital aortic valve stenosis--a retrospective multicenter survey of more than 1000 patients.

BACKGROUND: The value of balloon valvuloplasty of the aortic valve in childhood is still under debate. OBJECTIVE: To evaluate the results of the procedure in a retrospective multicenter survey of a large cohort over a long time interval. METHODS: Retrospective analysis of 1004 patients with balloon valvuloplasty of the aortic valve performed between 9/1985 and 10/2006 at 20 centers in Germany, Austria and Switzerland. Amongst others, the following parameters were evaluated before and after the procedure as well as at the end of follow-up or before surgery: clinical status, left ventricular function, transaortic pressure gradient, degree of aortic regurgitation, freedom from re-intervention or surgery. PATIENTS: Patients from 1 day to 18 years of age with aortic valve stenosis were divided into four groups: 334 newborns (1-28 days); 249 infants (29-365 days); 211 children (1-10 years), and 210 adolescents (10-18 years). RESULTS: Median follow-up was 32 months (0 days to 17.5 years). After dilatation the pressure gradient decreased from 65 (± 24)mm Hg to 26 (± 16)mm Hg and remained stable during follow-up. The newborns were the most affected patients. Approximately 60% of them had clinical symptoms and impaired left ventricular function before intervention. Complication rate was 15% in newborns, 11% in infants and 6% in older children. Independently of age, 50% of all patients were free from surgery 10 years after intervention. CONCLUSIONS: In this retrospective multicenter study, balloon valvuloplasty of the aortic valve has effectively postponed the need for surgery in infants, children and adolescents up to 18 years of age.

Transcatheter recanalization of acutely occluded modified systemic to pulmonary artery shunts in infancy.

BACKGROUND: There are few data on successful recanalization of acutely occluded modified aortopulmonary shunts in infants in whom the major component of pulmonary flow is shunt-dependent. PATIENTS AND METHODS: Seven infants (age range 6 days to 7 months) with modified (polytetrafluoroethylene) aortopulmonary shunts (central shunt n = 6, modified Blalock-Taussig shunt n = 1), in whom acute shunt occlusion was documented following surgery (interval from shunt surgery 2-6 days) underwent emergency cardiac catheterization with a view to reopening the shunt. Anterograde right ventricle to pulmonary artery flow was present in three patient; two other had additional aortopulmonary collateral vessels, and intravenous prostaglandin E (PGE1) infusion was restarted in three infants to achieve or maintain ductal patency. All occluded shunts could be crossed with a 4F endhole catheter and various guidewire combinations. Balloon dilation was performed using a balloon with the same nominal diameter as the shunt (5 or 6 mm). RESULTS: All shunts were successfully recanalized. Two infants with recurrent cyanotic episodes underwent stent implantation in the right ventricular outflow tract (RVOT) during the same procedure. One infant required shunt revision 24 h following balloon angioplasty, during which a small clot was evacuated from the distal end of the shunt. An immediate improvement in oxygen saturation could be documented in all patients. In the remaining six infants, shunt patency was confirmed at follow-up, until further definitive repair. One infant died of non-cardiac causes, while awaiting definitive repair. CONCLUSIONS: This small series demonstrates that acutely occluded shunts can be successfully recanalized, and that patency can be maintained until further definitive surgery in the majority of infants.

The diagnostic yield from implantable loop recorders in children and young adults.

BACKGROUND: Syncope and palpitations occur frequently in young patients. Noninvasive diagnostic testing may be inconclusive. AIM: To assess the diagnostic yield of implantable loop recorders in young patients. PATIENTS AND METHODS: Thirty-three young patients underwent implantation of a loop recorder for long-term monitoring of cardiac rhythm, to establish symptom-rhythm correlation. They belonged to one of three subgroups: those with structurally normal heart, normal electrocardiogram at rest, and negative family history (n = 16); patients with structural heart disease and previous surgical repair (n = 11), and patients with proven or suspected primary electrical disease (n = 6). A combination of automatic and patient-activated recordings was used to monitor cardiac rhythm during symptomatic episodes. RESULTS: There were no procedural complications. Diagnostic electrograms could be obtained in all patients. A high degree of symptom-rhythm correlation was established. In 8/33 patients, no recurrence of symptoms was observed either until end of battery life of the device (n = 4) or until last follow-up (n = 2). Specific cardiac therapy was required, based on rhythms recorded by the device in 15 patients (until last follow-up). This consisted of catheter ablation of a tachyarrhythmia (n = 7), pacemaker implantation or upgrade (n = 5) or ICD implantation (n = 5). In the remaining patients (n = 10), recurrence of symptoms was associated with a normal electrocardiogram, and in two of these patients a non-cardiac diagnosis was made. CONCLUSIONS: In selected patients, the implantable loop recorder provides valuable diagnostic information to guide further therapy.

The Amplatzer duct occluder for PDA closure: indications, technique of implantation and clinical outcome.

Following its introduction into clinical practice, the Amplatzer duct occluder (ADO) has achieved a definite place in the armamentarium of the interventional cardiologist for the closure of moderate to large sized PDAs. The device combines ease of use, including retrievability and repositioning when required, and a high occlusion rate (>99% complete occlusion of PDA within 6 months of implant, with the majority of occlusions occurring within 24 hours of implant). Possible complications, such as device embolization, protrusion of the retention disc of the device into the aorta producing aortic obstruction, or obstruction of a branch pulmonary artery by the device are also uncommon and can be avoided by choosing the appropriate sized device (with the pulmonary end of the device being 2mm larger in diameter than the minimum measured ductal diameter), and paying scrupulous attention to technique of deployment. The device can be safely deployed in infants >3.5 kg, and can currently close PDAs of upto 11 to 12mm in minimum diameter. A brief description of the device, the technique of implantation, and the clinical results to date are provided.

Experience with an ambulatory 12-lead Holter recording system for evaluation of pediatric dysrhythmias.

BACKGROUND: The 12-lead electrocardiogram demonstrates noninvasively many details concerning cardiac arrhythmias and their mechanism, but only for a limited period. A Holter system can record heart rhythm for a longer period, but shows 2 or 3 leads only. An ambulatory 12-lead Holter recording system combines the advantages of both. We report on our experience with the 12-lead Holter system in children. STUDY POPULATION: Twenty-four patients (age range, 3-22 years) underwent one or more 12-lead Holter recordings. Twelve patients had suspected or documented tachyarrhythmia, and 12 patients had suspected or proven long QT syndrome (LQTS). RESULTS: In the tachyarrhythmia group, 4 patients had supraventricular tachycardia and 7 ventricular tachycardia (VT). In the supraventricular tachycardia, group 1 patient had intra-atrial reentrant tachycardia with a single reentrant circuit, whereas another had multiple P-wave morphologies, suggesting multiple circuits. Two others had evidence for concealed atrioventricular accessory pathways. One patient had isolated supraventricular ectopy. In the VT group, 4 patients had uniform VT, and 3 patients had polymorphic VT. One patient with LQTS had macroscopic T-wave alternans. Two others showed intermittent extreme QT prolongation and T-wave notching during 12-lead Holter recording. THERAPEUTIC IMPLICATIONS: Patients with uniform VT underwent catheter ablation, guided in 2 instances by intracardiac pace mapping and comparison with the 12-lead Holter QRS morphology. All reentrant supraventricular arrhythmias with single P-wave morphology were ablated. One patient with intra-atrial reentrant tachycardia and multiple circuits received an antitachycardia pacemaker. In the LQTS group, the neonate with T-wave alternans received mexiletine in addition to beta-blocker therapy. CONCLUSION: In individual patients, the 12-lead Holter system provides important additional information about the arrhythmia and helps to plan appropriate therapy. Intermittent T-wave morphology changes can be diagnosed using 12-lead Holter recordings in patients with LQTS, allowing dynamic T-wave changes to be monitored. In specific cases, this may help identify patients with LQTS and also influence pharmacological therapy.

Transhepatic approach for catheter interventions in infants and children with congenital heart disease.

UNLABELLED: We report on our experience with transhepatic access for catheter interventions in six children (age range 2.5 months-9 years). Three had systemic venous anomalies, and one infant a femoral venous occlusion. In two further patients with bradyarrhythmia after a Fontan operation with an intraatrial Gore-Tex tunnel, transhepatic access was chosen to achieve a perpendicular orientation of the transseptal needle to the atrial baffle, allowing puncture of the Gore-Tex membrane. Two of the patients underwent ablation of an accessory pathway; in one an atrial septal defect was closed. A 2.5 month old baby after Norwood I operation, underwent balloon dilation of the pulmonary arteries. Two patients after prior Fontan surgery underwent DDDR pacemaker implantation. The size of the introducer sheath ranged from 4 F up to two 9 F introducers in the same vein for pacemaker insertion. At the end of the procedure, hemostasis was achieved by external compression. RESULTS: Transhepatic access could be established in all six patients (using a mirror image approach in children with left atrial isomerism) and the interventional procedures could be performed as planned. In one patient with implantation of a permanent pacemaker, a subcutaneous hematoma occurred, requiring blood transfusion. CONCLUSION: In selected pediatric patients, transhepatic access for catheter intervention can easily be achieved.