Editorial
Arrhythmias in Adult Congenital Heart Disease
Looking at the title of this edition of Herzschrittmachertherapie und Elektrophysiologie, the reader may ask if it may contribute anything relevant to coping with his or her most likely already quite timewise challenging clinical routine. Of course, we would like to encourage you to continue reading it (and not to put it aside after scanning the topic list). And this is not only because of us (as the authors and editors) being particularly passionate about these topics! It is mostly, because we are very much aware that as a medicine or cardiology specialist consultant, you are periodically exposed to patients with adult congenital heart disease (ACHD). Sometimes this may be as a non-dominant diagnosis when dealing with a completely different question or as the main cause of the presenting clinical symptoms.
In future, patient contacts like this will be more likely to happen for colleagues not necessarily specialized in ACHD, as the number of patients reaching adulthood with congenital heart disease (due to the advances in diagnostics, but also in the pharmacologic, interventional and surgical therapies) in the last 3-4 decades has dramatically increased in the developed countries. And a large number of these adults are not necessarily followed at specialized centres. Current estimates count about 1.8 million adults in Europe with ACHD (with about 200.000 in Germany alone) which thereby has overtaken the number of children (below the age of 18 yrs) with congenital heart disease since 2005 (Source: National register for angeboreneHerzfehlere.V.).
Patient, even with “mild” ACHD, do frequently need constant medical support due to the variable consequences of the cardiac anomaly, even if it has been interventionally or surgically treated. Therefore, the medical follow-up of these patients has already been part of the “conventional” adult cardiology, even with the absolute number still relatively low and with immense heterogeneity with regards to characteristics and condition of the CHD.
Beside the “attractiveness” of an initially welcoming difference from the clinical routine cases, there may later be a component of certain unease when clinical presentations and necessary therapeutic options are more remote from the conventional options, which are routinely applied at adults with “normal” cardiovascular anatomy.
Arrhythmias is the lead clinical problem during long term follow-up of patients with ACHD with consequences on the whole spectrum of quality of life, morbidity and mortality. They present in more than 50% of the cases the need for inpatient or outpatient review, mostly because they result in reduced function of the systemic ventricle leading to heart failure.
The vast majority of brady- and tachycardic arrhythmias in normal adults is well understood and the majority is well controlled or even curable using modern techniques. Patients with ACHD present with the same arrhythmias, but in a completely different anatomical context and possibly with more frequent combinations of various types of arrhythmias. This makes the non-invasive and invasive diagnostic far more challenging, as well as all therapeutic measures which may be applicable only in specialised centres for ACHD.
In this edition of Herzschrittmachertherapie und Elektrophysiologie, we present the major topics of arrhythmia and electrophysiology in adults with CHD to provide an overview on the therapeutic options in the respective conditions with the aim to assist with the occasionally necessary collaboration of patients with ACHD and to highlight the options of specialised follow-up.
Kristina Wasmer from the group of Gerhard Diller and Lars Eckhardt, Muenster summarized the incidence of arrhythmias in ACHD with regards of the specific substrates and arrhythmia mechanisms, as well as the potential underlying arrhythmogenic causes by chronic cardiac strain or as a consequence of the surgical intervention.
The overview on opportunities of acute and longterm management of brady- and tachycardic arrhythmias in ACHD is presented by Gabriele Hessling from Munich. The article covers the treatment in the emergency situation of hemodynamically significant or non-tolerated arrhythmia, general considerations for short- or even longterm medical or interventional treatment and considers potential surgical options for both arrhythmia and hemodynamic optimisation.
The very complex topic of permanent device implantation for anti-bradycardic, anti-tachycardic and biventricular devices is present in 3 consecutive chapters. The anti-bradycardic pacemaker implantation with special emphasis on optimal device choice, programming and positioning of the leads (endoepicardial) is presented by Peter Zartner, St. Augustin. H. Chubb and Eric Rosenthal, London, UK concentrate on the pecularities of ICD implantation in ACHD patients, ranging from the indication to the technical challenges due to vascular or intracardial obstacles. Jan Janoussek, Prag, Tchech Republic, explains in the last of these 3 chapters, if and how resynchronisation can be employed even in complex ACHD conditions, and if and when this makes sense in the first place.
Invasive interventional electrophysiology procedures are nowadays even in the presence of complex congenital anomalies possible. With the recent progress in arrhythmia management, this has also very successfully been applied to this patient cohort, as their hemodynamic tolerance is often less good whilst medical therapy is even less reliable.
The state of the art in invasive electrophysiology in ACHD is divided into 3 chapters: the group of Sabine Ernst and Yen Ho in London, UK focuses on atrial tachycardias which appear in up 50% of all patients with ACHD during longterm follow-up. Katja Zeppenfeld from Leiden, The Netherlands, provides an overview on the chances and limitations of catheter ablation of ventricular tachycardias. The interventional options in the remaining group of supraventricular tachycardias on the basis of congenital or ACHD-associated substrates is presented by Joachim Hebe, Bremen.
Hideki Uemura, London (UK) is reflectingfrom a surgeon’s perspective on which extent the growing understanding of the invasive electrophysiologic information is already manifested in the initial surgical procedure planning and any following further surgical modification.
In the hope, the this edition will not only be a welcomed distraction from the also quite interesting but also frequently repeated topics of larger patient cohorts (such as atrial fibrillation or coronary artery disease), we wish you on behalf of all the other authors many insights, stimulation and certainly enjoyment with the reading of this issue.
Yours sincerely,
Joachim Hebe and Sabine Ernst
Corresponding address
Dr. med. Joachim Hebe
Zentrum f. Elektrophysiologie Bremen
Am Klinikum Links der Weser
Sen.-Weßling-Str. 1
28277 Bremen, Germany
Dr. Sabine Ernst
Research Lead Electrophysiology
Royal Brompton Hospital
Sydney Street
London
SW3 6NP
England
Conflict of interest
J. Hebe and S. Ernst state that there is no conflict of interest.