We present a 7-year-old boy with tuberous sclerosis and congenital segmental lymphedema (CSL) of ... more We present a 7-year-old boy with tuberous sclerosis and congenital segmental lymphedema (CSL) of the left leg, as well as two aortic aneurysms. He was treated with everolimus (EVE) since the age of 14 months. His CSL regressed under treatment with EVE. His first aneurysms required operative intervention at age of 17 months. Four months afterward a new aortic aneurysm had been detected above the Dracon graft, but this one remained stable since that time. The patient didn't experience severe side effects. EVE has been well tolerated without disturbance of somatic growth or serious adverse effect.
Einleitung: Die Anwendung der Single Incision Laparoscopic Surgery (SILS)-Methode als Weiterentwi... more Einleitung: Die Anwendung der Single Incision Laparoscopic Surgery (SILS)-Methode als Weiterentwicklung der bereits in vielen chirurgischen Teilbereichen etablierten laparoskopischen Technik findet in der Chirurgie inzwischen regelhaft Anwendung. Innerhalb der letzten Jahre werden zunehmend auch kinderchirurgische[zum vollständigen Text gelangen Sie über die oben angegebene URL]
Das Wissen uber die pathophysiologischen Vorgange bei Herzrhythmusstorungen bei Kindern und Jugen... more Das Wissen uber die pathophysiologischen Vorgange bei Herzrhythmusstorungen bei Kindern und Jugendlichen hat sich in den letzten Jahren rasant erweitert. Bei padiatrischen Patienten mit Tachykardien haben sich die elektrophysiologische Untersuchung und Katheterablation als auserst effektive Verfahren erwiesen. Bei Neugeborenen und Sauglingen kommt es dagegen im Laufe des 1. Lebensjahres bei > 90% der Patienten zu einem spontanen Sistieren der supraventrikularen Tachykardien, so dass ein abwartendes Vorgehen mit medikamentoser Therapie sinnvoll ist. In dem folgenden Beitrag werden funf wichtige Publikationen der letzten 12 Monate vorgestellt. Anhand der kurzlich aktualisierten Leitlinien werden die Empfehlungen zur Vorgehensweise bei asymptomatischen Kindern mit einem Praexzitationssyndrom dargestellt. In der medikamentosen Therapie von supraventrikularen Tachykardien im ersten Lebensjahr zeigte eine randomisierte Doppelblindstudie keinen Unterschied von Digoxin versus Propranolol. Durch molekulargenetische Untersuchungen und klinische Studien konnte die Diagnostik und Therapie von Ionenkanalerkrankungen verbessert werden, wie z.B. bei Mutationen im SCN5A-Gen. Bei padiatrischen Patienten mit einem Long-QT Syndrom oder einer catecholaminergen polymorphen ventrikularen Tachykardie fuhrte die Sympathektomie bei lebensbedrohlichen ventrikularen Tachykardien zu einer deutlichen Reduktion der Arrhythmieereignisse. Insbesondere bei Ionenkanalerkrankungen wurde in den letzten Jahren in zunehmender Anzahl ein Kardioverter-Defibrillator auch zur Sekundarprophylaxe implantiert. Die internationalen Leitlinien zur Implantation wurden erst kurzlich aktualisiert. Bibliography: [1] Cohen MI, Triedman JK, Cannon BC, et al; Pediatric and Congenital Electrophysiology Society (PACES); Heart Rhythm Society (HRS); American College of Cardiology Foundation (ACCF); American Heart Association (AHA); American Academy of Pediatrics (AAP); Canadian Heart Rhythm Society (CHRS). PACES/HRS expert consensus statement on the management of the asymptomatic young patient with a Wolff-Parkinson-White (WPW, ventricular preexcitation) electrocardiographic pattern: developed in partnership between the Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS). Endorsed by the governing bodies of PACES, HRS, the American College of Cardiology Foundation (ACCF), the American Heart Association (AHA), the American Academy of Pediatrics (AAP), and the Canadian Heart Rhythm Society (CHRS). Heart Rhythm 2012;9(6):1006–1024 [2] Sanatani S, Potts JE, Reed JH, et al. The study of antiarrhythmic medications in infancy (SAMIS): a multicenter, randomized controlled trial comparing the efficacy and safety of digoxin versus propranolol for prophylaxis of supraventricular tachycardia in infants. Circ Arrhythm Electrophysiol 2012;5(5):984–991 [3] Chockalingam P, Clur SA, Breur JM, et al. The diagnostic and therapeutic aspects of loss-of-function cardiac sodium channelopathies in children. Heart Rhythm 2012;9(12):1986–1992 [4] Schneider HE, Steinmetz M, Krause U, Kriebel T, Ruschewski W, Paul T. Left cardiac sympathetic denervation for the management of life-threatening ventricular tachyarrhythmias in young patients with catecholaminergic polymorphic ventricular tachycardia and long QT syndrome. Clin Res Cardiol 2013;102(1):33–42 [5] Russo AM, Stainback RF, Bailey SR, et al. ACCF/HRS/AHA/ASE/HFSA/SCAI/SCCT/SCMR 2013 appropriate use criteria for implantable cardioverter-defibrillators and cardiac resynchronization therapy: a report of the American College of Cardiology Foundation appropriate use criteria task force, Heart Rhythm Society, American Heart Association, American Society of Echocardiography, Heart Failure Society of America, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, and Society for Cardiovascular Magnetic Resonance. J Am Coll Cardiol 2013;61(12):1318–1368
The following document provides a summary of the guidelines and recommendations for paediatric ca... more The following document provides a summary of the guidelines and recommendations for paediatric cardiac intensive care training as a requirement for recognition as a European paediatric cardiologist. It is therefore primarily targeting paediatric cardiology trainees in Europe, including those doctors who might wish to become experts in cardiac intensive care. These recommendations represent a frame for consistency, will evolve, and may be adapted to specific institutional requirements. They will be complemented by a learning module to be provided by our Association in the near future.
Bibliografische Information der Deutschen Nationalbibliothek Die Deutsche Nationalbibliothek verz... more Bibliografische Information der Deutschen Nationalbibliothek Die Deutsche Nationalbibliothek verzeichnet diese Publikation in der Deutschen Nationalbibliografie; detaillierte bibliografische Daten sind im Internet über http://dnb.d-nb.de abrufbar.
Circulation-arrhythmia and Electrophysiology, Nov 1, 2016
C atheter ablation of the slow conducting pathway (SP) of the atrioventricular (AV) node is the r... more C atheter ablation of the slow conducting pathway (SP) of the atrioventricular (AV) node is the recommended treatment for AV nodal reentrant tachycardia (AVNRT) in children and adults. With both radiofrequency and cryoenergy, high procedural success rates of 98% to 100% have been reported. 1,2 In adult patients, freedom from AVNRT has been achieved in 95% after radiofrequency ablation and in 90% after the use of cryoenergy 1 year after the initial ablation procedure. 3 In children and adolescents, data from the pediatric radiofrequency catheter ablation registry acquired more than a decade ago reported a significant number of recurrences with 71% freedom from AVNRT 3 years post ablation. 4 However, recent follow-up studies of pediatric patients after AVNRT ablation reported freedom from AVNRT between 84% and 100% with radiofrequency 2,5 and between 78% and 93% with cryoenergy, 6-8 respectively. These studies on a limited number of pediatric patients analyzed data on the follow-up of 1 to 3 years post ablation. Little information on the course beyond 3 years after AVNRT ablation in pediatric patients has been published. The purpose of the present study was to evaluate the longterm course of pediatric patients after AVNRT ablation at our institution. Foci of interest were (1) impact of the primary procedural end point (SP ablation versus SP modulation) on long-term success, (2) impact of body weight on long-term safety and success, (3) incidence and timing of late AVNRT recurrence, and (4) incidence of late AV block or new tachyarrhythmias attributable to AVNRT ablation. See Editorial by Kirsh Methods Patients Between October 2002 and May 2014, a total of 249 children and adolescents <18 years of age had undergone catheter ablation for AVNRT at our institution. Procedural data and primary success rates and acute complications have recently been published. 1 Patients, in whom AVNRT ablation failed (n=5), were not enrolled into the present study: although a repeat ablation was offered for all 5 subjects with procedural failure, 3 out of 5 patients were lost during follow-up and 1 individual decided against a repeat procedure. Additionally, 3 patients with permanent AV block immediately after radiofrequency ablation (n=3) 1 were excluded. Finally, follow-up data ≥1 year after AVNRT ablation of 241 patients were included into this study. Follow-up data of patients after primary AVNRT ablation procedures were exclusively considered to avoid bias by including follow-up
Herzschrittmachertherapie Und Elektrophysiologie, Nov 9, 2021
Chronische Tachykardien mit schmalen QRS-Komplexen können auch bei einer langsamen Tachykardiefre... more Chronische Tachykardien mit schmalen QRS-Komplexen können auch bei einer langsamen Tachykardiefrequenz zu einer Tachykardie-induzierten Kardiomyopathie führen, insbesondere bei zusätzlichem Vorliegen eines begleitenden Herzfehlers. Häufig ist eine medikamentöse antiarrhythmische Therapie insuffizient, sodass eine Ablation des anatomischen Substrates erforderlich ist. Nach erfolgreicher Ablation kommt es erfreulicherweise in der Regel zu einer deutlichen Verbesserung bzw. Normalisierung der Ventrikelfunktion.
ABSTRACT Die arrhythmogene rechtsventrikuläre Dysplasie/Cardiomyopathie (ARVD/C) ist eine seltene... more ABSTRACT Die arrhythmogene rechtsventrikuläre Dysplasie/Cardiomyopathie (ARVD/C) ist eine seltene, aber bedeutsame Ursache für den plötzlichen Herztod bei Jugendlichen und jungen Erwachsenen. Das sehr unterschiedliche Erscheinungsbild dieser Erkrankung im Kindesalter mit den Problemen der Diagnosestellung wird am Beispiel von 2Patienten beschrieben. Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a rare, but important cause for sudden death in adolescents and young adults. Part of the patients affected show the pattern of autosomal-dominant inheritance. Two pediatric patients with ARVD/C are presented who may reflect the spectrum of clinical presentation of ARVD/C in childhood resulting in difficulties or even delay to establish the correct diagnosis. One patient with a sporadic form of ARVD/C presented with a syncope and spontaneous as well as inducible ventricular tachycardia. On the ECG, an epsilon wave could be identified. An automatic cardioverter defibrillator was implanted. The second patient had a familiar form of ARVD/C with no symptoms. There was a history of frequent sudden deaths in this family. Biopsies of the right ventricular myocardium showed fibrosis with deposition of fatty tissue. There was clear evidence of ARVD/C in the necropsy of the patient‘s aunt. Therapy with propanolol was started in this patient.
We present a 7-year-old boy with tuberous sclerosis and congenital segmental lymphedema (CSL) of ... more We present a 7-year-old boy with tuberous sclerosis and congenital segmental lymphedema (CSL) of the left leg, as well as two aortic aneurysms. He was treated with everolimus (EVE) since the age of 14 months. His CSL regressed under treatment with EVE. His first aneurysms required operative intervention at age of 17 months. Four months afterward a new aortic aneurysm had been detected above the Dracon graft, but this one remained stable since that time. The patient didn't experience severe side effects. EVE has been well tolerated without disturbance of somatic growth or serious adverse effect.
Einleitung: Die Anwendung der Single Incision Laparoscopic Surgery (SILS)-Methode als Weiterentwi... more Einleitung: Die Anwendung der Single Incision Laparoscopic Surgery (SILS)-Methode als Weiterentwicklung der bereits in vielen chirurgischen Teilbereichen etablierten laparoskopischen Technik findet in der Chirurgie inzwischen regelhaft Anwendung. Innerhalb der letzten Jahre werden zunehmend auch kinderchirurgische[zum vollständigen Text gelangen Sie über die oben angegebene URL]
Das Wissen uber die pathophysiologischen Vorgange bei Herzrhythmusstorungen bei Kindern und Jugen... more Das Wissen uber die pathophysiologischen Vorgange bei Herzrhythmusstorungen bei Kindern und Jugendlichen hat sich in den letzten Jahren rasant erweitert. Bei padiatrischen Patienten mit Tachykardien haben sich die elektrophysiologische Untersuchung und Katheterablation als auserst effektive Verfahren erwiesen. Bei Neugeborenen und Sauglingen kommt es dagegen im Laufe des 1. Lebensjahres bei > 90% der Patienten zu einem spontanen Sistieren der supraventrikularen Tachykardien, so dass ein abwartendes Vorgehen mit medikamentoser Therapie sinnvoll ist. In dem folgenden Beitrag werden funf wichtige Publikationen der letzten 12 Monate vorgestellt. Anhand der kurzlich aktualisierten Leitlinien werden die Empfehlungen zur Vorgehensweise bei asymptomatischen Kindern mit einem Praexzitationssyndrom dargestellt. In der medikamentosen Therapie von supraventrikularen Tachykardien im ersten Lebensjahr zeigte eine randomisierte Doppelblindstudie keinen Unterschied von Digoxin versus Propranolol. Durch molekulargenetische Untersuchungen und klinische Studien konnte die Diagnostik und Therapie von Ionenkanalerkrankungen verbessert werden, wie z.B. bei Mutationen im SCN5A-Gen. Bei padiatrischen Patienten mit einem Long-QT Syndrom oder einer catecholaminergen polymorphen ventrikularen Tachykardie fuhrte die Sympathektomie bei lebensbedrohlichen ventrikularen Tachykardien zu einer deutlichen Reduktion der Arrhythmieereignisse. Insbesondere bei Ionenkanalerkrankungen wurde in den letzten Jahren in zunehmender Anzahl ein Kardioverter-Defibrillator auch zur Sekundarprophylaxe implantiert. Die internationalen Leitlinien zur Implantation wurden erst kurzlich aktualisiert. Bibliography: [1] Cohen MI, Triedman JK, Cannon BC, et al; Pediatric and Congenital Electrophysiology Society (PACES); Heart Rhythm Society (HRS); American College of Cardiology Foundation (ACCF); American Heart Association (AHA); American Academy of Pediatrics (AAP); Canadian Heart Rhythm Society (CHRS). PACES/HRS expert consensus statement on the management of the asymptomatic young patient with a Wolff-Parkinson-White (WPW, ventricular preexcitation) electrocardiographic pattern: developed in partnership between the Pediatric and Congenital Electrophysiology Society (PACES) and the Heart Rhythm Society (HRS). Endorsed by the governing bodies of PACES, HRS, the American College of Cardiology Foundation (ACCF), the American Heart Association (AHA), the American Academy of Pediatrics (AAP), and the Canadian Heart Rhythm Society (CHRS). Heart Rhythm 2012;9(6):1006–1024 [2] Sanatani S, Potts JE, Reed JH, et al. The study of antiarrhythmic medications in infancy (SAMIS): a multicenter, randomized controlled trial comparing the efficacy and safety of digoxin versus propranolol for prophylaxis of supraventricular tachycardia in infants. Circ Arrhythm Electrophysiol 2012;5(5):984–991 [3] Chockalingam P, Clur SA, Breur JM, et al. The diagnostic and therapeutic aspects of loss-of-function cardiac sodium channelopathies in children. Heart Rhythm 2012;9(12):1986–1992 [4] Schneider HE, Steinmetz M, Krause U, Kriebel T, Ruschewski W, Paul T. Left cardiac sympathetic denervation for the management of life-threatening ventricular tachyarrhythmias in young patients with catecholaminergic polymorphic ventricular tachycardia and long QT syndrome. Clin Res Cardiol 2013;102(1):33–42 [5] Russo AM, Stainback RF, Bailey SR, et al. ACCF/HRS/AHA/ASE/HFSA/SCAI/SCCT/SCMR 2013 appropriate use criteria for implantable cardioverter-defibrillators and cardiac resynchronization therapy: a report of the American College of Cardiology Foundation appropriate use criteria task force, Heart Rhythm Society, American Heart Association, American Society of Echocardiography, Heart Failure Society of America, Society for Cardiovascular Angiography and Interventions, Society of Cardiovascular Computed Tomography, and Society for Cardiovascular Magnetic Resonance. J Am Coll Cardiol 2013;61(12):1318–1368
The following document provides a summary of the guidelines and recommendations for paediatric ca... more The following document provides a summary of the guidelines and recommendations for paediatric cardiac intensive care training as a requirement for recognition as a European paediatric cardiologist. It is therefore primarily targeting paediatric cardiology trainees in Europe, including those doctors who might wish to become experts in cardiac intensive care. These recommendations represent a frame for consistency, will evolve, and may be adapted to specific institutional requirements. They will be complemented by a learning module to be provided by our Association in the near future.
Bibliografische Information der Deutschen Nationalbibliothek Die Deutsche Nationalbibliothek verz... more Bibliografische Information der Deutschen Nationalbibliothek Die Deutsche Nationalbibliothek verzeichnet diese Publikation in der Deutschen Nationalbibliografie; detaillierte bibliografische Daten sind im Internet über http://dnb.d-nb.de abrufbar.
Circulation-arrhythmia and Electrophysiology, Nov 1, 2016
C atheter ablation of the slow conducting pathway (SP) of the atrioventricular (AV) node is the r... more C atheter ablation of the slow conducting pathway (SP) of the atrioventricular (AV) node is the recommended treatment for AV nodal reentrant tachycardia (AVNRT) in children and adults. With both radiofrequency and cryoenergy, high procedural success rates of 98% to 100% have been reported. 1,2 In adult patients, freedom from AVNRT has been achieved in 95% after radiofrequency ablation and in 90% after the use of cryoenergy 1 year after the initial ablation procedure. 3 In children and adolescents, data from the pediatric radiofrequency catheter ablation registry acquired more than a decade ago reported a significant number of recurrences with 71% freedom from AVNRT 3 years post ablation. 4 However, recent follow-up studies of pediatric patients after AVNRT ablation reported freedom from AVNRT between 84% and 100% with radiofrequency 2,5 and between 78% and 93% with cryoenergy, 6-8 respectively. These studies on a limited number of pediatric patients analyzed data on the follow-up of 1 to 3 years post ablation. Little information on the course beyond 3 years after AVNRT ablation in pediatric patients has been published. The purpose of the present study was to evaluate the longterm course of pediatric patients after AVNRT ablation at our institution. Foci of interest were (1) impact of the primary procedural end point (SP ablation versus SP modulation) on long-term success, (2) impact of body weight on long-term safety and success, (3) incidence and timing of late AVNRT recurrence, and (4) incidence of late AV block or new tachyarrhythmias attributable to AVNRT ablation. See Editorial by Kirsh Methods Patients Between October 2002 and May 2014, a total of 249 children and adolescents <18 years of age had undergone catheter ablation for AVNRT at our institution. Procedural data and primary success rates and acute complications have recently been published. 1 Patients, in whom AVNRT ablation failed (n=5), were not enrolled into the present study: although a repeat ablation was offered for all 5 subjects with procedural failure, 3 out of 5 patients were lost during follow-up and 1 individual decided against a repeat procedure. Additionally, 3 patients with permanent AV block immediately after radiofrequency ablation (n=3) 1 were excluded. Finally, follow-up data ≥1 year after AVNRT ablation of 241 patients were included into this study. Follow-up data of patients after primary AVNRT ablation procedures were exclusively considered to avoid bias by including follow-up
Herzschrittmachertherapie Und Elektrophysiologie, Nov 9, 2021
Chronische Tachykardien mit schmalen QRS-Komplexen können auch bei einer langsamen Tachykardiefre... more Chronische Tachykardien mit schmalen QRS-Komplexen können auch bei einer langsamen Tachykardiefrequenz zu einer Tachykardie-induzierten Kardiomyopathie führen, insbesondere bei zusätzlichem Vorliegen eines begleitenden Herzfehlers. Häufig ist eine medikamentöse antiarrhythmische Therapie insuffizient, sodass eine Ablation des anatomischen Substrates erforderlich ist. Nach erfolgreicher Ablation kommt es erfreulicherweise in der Regel zu einer deutlichen Verbesserung bzw. Normalisierung der Ventrikelfunktion.
ABSTRACT Die arrhythmogene rechtsventrikuläre Dysplasie/Cardiomyopathie (ARVD/C) ist eine seltene... more ABSTRACT Die arrhythmogene rechtsventrikuläre Dysplasie/Cardiomyopathie (ARVD/C) ist eine seltene, aber bedeutsame Ursache für den plötzlichen Herztod bei Jugendlichen und jungen Erwachsenen. Das sehr unterschiedliche Erscheinungsbild dieser Erkrankung im Kindesalter mit den Problemen der Diagnosestellung wird am Beispiel von 2Patienten beschrieben. Arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) is a rare, but important cause for sudden death in adolescents and young adults. Part of the patients affected show the pattern of autosomal-dominant inheritance. Two pediatric patients with ARVD/C are presented who may reflect the spectrum of clinical presentation of ARVD/C in childhood resulting in difficulties or even delay to establish the correct diagnosis. One patient with a sporadic form of ARVD/C presented with a syncope and spontaneous as well as inducible ventricular tachycardia. On the ECG, an epsilon wave could be identified. An automatic cardioverter defibrillator was implanted. The second patient had a familiar form of ARVD/C with no symptoms. There was a history of frequent sudden deaths in this family. Biopsies of the right ventricular myocardium showed fibrosis with deposition of fatty tissue. There was clear evidence of ARVD/C in the necropsy of the patient‘s aunt. Therapy with propanolol was started in this patient.
Uploads
Papers by Thomas Kriebel