Rhythmology for congenital heart defects

The incidence of cardiac arrhythmias in children is around 10% for benign arrhythmias and up to 1% for arrhythmias that potentially require treatment. Irregularities in the heartbeat are often incidental findings in paediatric practice, just as asymptomatic WPW syndrome is often detected during screening ECGs.

Tachycardia usually occurs at school age as paroxysmal supraventricular tachycardia due to a congenital accessory pathway or dual AV nodal conduction. In young infants, permanent, relatively slow tachycardias with frequencies below 200/min can lead to tachymyopathy, which has a good prognosis with appropriate antiarrhythmic treatment.

If syncope occurs during exercise, an arrhythmogenic cause is suspected, whereas bradycardia is usually the result of myocarditis or congenital AV block, but is rare overall. In structural heart disease, on the other hand, associated cardiac arrhythmias are frequently found.

The symptoms of a cardiac arrhythmia depend not only on the type of arrhythmia, but also on the possible presence of structural heart disease. The recording of a 12-lead ECG is the method of choice for establishing the diagnosis; further diagnostics are carried out using long-term ECG recording, external event recorders, stress ECG examinations and, in rare cases, an electrophysiological examination. Further cardiac diagnostics using echocardiography, MRI or cardiac catheterisation are used to detect associated congenital or acquired vitia or coronaropathies.

According to the anatomical and rhythmological findings, the therapy is not only based on the ECG diagnosis, but above all on the haemodynamic effects of the arrhythmia, the frequency of occurrence and the potential arrhythmogenic risk: The aim of acute therapy is to quickly restore an adequate circulatory situation using medication, temporary pacemakers or cardioversion / defibrillation. Long-term therapy is used to prevent recurrence (medication) or for definitive therapy: catheter ablation or permanent implantation of pacemakers or ICDs.

An electrophysiological examination is an invasive  catheter examination to definitively clarify the specific tachycardia; under analgosedation or anaesthesia (rare), several catheters are placed via the femoral veins into the various heart chambers and the local electrical signals are recorded and analysed. Thanks to the use of so-called "3-dimensional mapping procedures", this examination is now usually carried out with minimal or even no X-rays at all.

In radiofrequency current ablation, the tachycardia substrate is obliterated by heating the  myocardium to 50-60°C; in the case of anatomical proximity to the sinus or AV node, obliteration by cold (so-called cryoablation) is often used instead of tissue necrosis by means of radiofrequency current,

Pacemaker surgery is indicated for symtpomatic, non-reversible  bradycardias: In small children ( <10 kg body weight), it is usually performed epicardially (preferably on the left ventricle), later rather transvenously endocardially via the subclavian vein. In infancy, single-chamber (VVI) pacemaker implantations are predominantly performed with transvenous access, even in the case of AV block, in order to protect the veins.

An indication for implantation of an internal cardioverter/defibrillator (ICD) exists for life-threatening ventricular arrhythmias.

Specialist consultation

Special consultations for patients with cardiac arrhythmia and pacemaker patients are available by appointment at kika-ambulanz@dhzc-charite.de during the consultation hours of our outpatient clinic.

heart failure. We also have particular expertise in the care of EMAH with cardiac arrhythmias, pregnant EMAH patients and patients with pulmonary hypertension associated with a congenital heart defect.

Contact: Prof. Dr Oktay Tutarel, Head of the EMAH Centre

Further information

One of the main focuses of our clinic is the treatment of cardiac arrhythmias with pacemakers and implantable defibrillators in children and in the case of congenital heart defects. We offer individualised therapeutic approaches and state-of-the-art treatment procedures. Our aim is to provide our patients with lifelong complication-free care.

Special consultations for patients with cardiac arrhythmia and pacemaker patients are available by appointment at kika-ambulanz@dhzc-charite.de during the consultation hours of our outpatient clinic.

Further information

Fine obstetric diagnostics are now used to detect both bradycardic cardiac arrhythmias (usually congenital AV block) and tachycardic cardiac arrhythmias at an early stage. In cooperation with the rhythmology department at the Clinic for Obstetrics, prenatal counselling and therapy is provided at an early stage in order to establish the best possible treatment strategies in cooperation with the patient.

In cooperation and under the expertise of our Professor of Cardiac Genetics, Prof. Dr. Sabine Klaassen, individualised genetic counselling, diagnostics and prognostic assessment of the findings for specific therapy is carried out for potentially genetically determined arrhythmia syndromes such as hypertrophic cardiomyopathy, long QT syndrome, catecholaminergic polymorphic ventricular tachycardia, Brugada syndrome or other rare diseases.

We care for paediatric patients from the entire Charité Children's Hospital as well as adults with congenital heart defects. We work closely with our colleagues at the DHZC cardiology clinics on the Virchow-Klinikum Campus, Charité Mitte Campus and Benjamin Franklin Campus to provide this consultative care.

We also offer consultative care for external patients who are referred to us by paediatric cardiologists in private practice and clinics in Berlin, Brandenburg, Mecklenburg-Western Pomerania and other national and international referring physicians.

We work closely with colleagues from the Clinic for Surgery of Congenital Heart Defects -Paediatric Heart Surgery on these topics:

• invasive intraoperative ablation therapy,
• epicardial device therapy,
• ablation under ECMO.

In certain cardiac surgery procedures for patients with AHF, such as Ebstein's anomaly, individualised preoperative risk stratification is performed using electrophysiological testing. If patients who require cardiac surgery, such as tetralogy of Fallot, also have tachycardic arrhythmias, these can be surgically ablated during cardiac surgery.  

In complex, life-threatening arrhythmias, ablation is also performed transthoracic epicardially interventional and in rare cases of haemodynamic instability, ablation is also performed using extracorporeal membrane oxygenation (ECMO).

For over 15 years, we have been offering left cervical sympathetic denervation (LCSD) in collaboration with the Department of Paediatric Surgery at Charité, senior physician Dr Axel Schneider. In this procedure, adrenergic nerve impulses are suppressed, which can lead to life-threatening arrhythmias in certain rare arrhythmogenic diseases such as LQTS or CPVT. The procedure is performed under anaesthetic and is minimally invasive thoracoscopic.

We treat patients with cardiac arrhythmias as inpatients and day patients.

Certain procedures, such as electrical cardioversions, transoesophageal electrophysiological examinations or electrophysiological examinations via implanted devices as well as the implantation of event recorders, are usually performed on a day-case basis.

Currently, ablation therapy and the implantation of pacemakers and ICDs are usually performed on an inpatient basis and are associated with at least one overnight stay in hospital; as a rule, a parent can of course also be admitted.

• Clinical examination incl. RR measurement, SO2 monitoring, echocardiography
• 12-channel ECG, 24-72 h LZ ECG, 12-channel LZ ECG
• External event recorder
• Exercise ergometry / spiroerometry
  • Treadmill
  • Ergometer
• Tilt table examination
• Electrophysiological examinations
  • Non-invasive diagnostic
    • transoesophageal
    • via device (SM, ICD)
    • Mediakment tests (adrenaline, ajmaline, others)
  • invasive diagnostic
    • conventional
    • 3 D mapping
      • conventional (NavX Ensite X Precision)
      • high resolution (NavX Ensite X Voxel)
  • therapeutic
    • Cryoablation (focal)
    • RF ablation
      • conventional (RFA)
      • cooled (iRFA)
      • Pressure sensor-controlled
      • endocardial / epicardial
    • Pulse field ablation
  • operative             
    • implantable event recorders
    • 1, 2 and 3 chamber pacemakers / conduction system pacing
    • 1,2 and 3 chamber ICD
    • S-ICD
    • Probe extraction
    • LCSD (OA Dr A. Schneider, Paediatric Surgery, Charité)