Mapping of Atrial Arrhythmias in Complex Congenital Heart Disease
Mapping of Atrial Arrhythmias in Complex Congenital Heart Disease
Published: November 2007
Advances in the treatment of complex congenital heart disease over the past 20 years have led to a dramatic improvement in long-term survival.1 However, the incidence of cardiac arrhythmias as a sequel of palliative heart surgery has also increased significantly in adolescent and adult survivors of congenital heart disease surgery.1,2 Cardiac arrhythmias are the leading cause of emergency admissions in adult congenital heart disease patients with or without surgical intervention.3
The incidence of late arrhythmias has been associated with long-term abnormal pressure–volume load, hypoxia, surgically created scars or suture lines.4–6 Residual haemodynamic problems are frequently encountered in these patients and may be aggravated by the arrhythmia, resulting in heart failure, acute collapse or cardiac arrest. The risk of sudden death is reported to be as high as 6–10% in long-term survivors after surgery for congenital heart disease.1
As patients with congenital heart disease form a heterogeneous population, an optimal treatment strategy for the individual patient has to be defined. This may include medical therapy, catheter ablation or implantation of a pacemaker or automatic internal defibrillator. Medical therapy is often insufficient to control the arrhythmia and exposes patients to the risk of serious side effects, especially with long-term treatment.7 Catheter ablation offers an alternative treatment strategy in many of these patients. New mapping and ablation techniques have improved the understanding of complex arrhythmia mechanisms.
There are some crucial steps to take when approaching an ablation therapy in patients with congenital heart disease. These include adequate preparation of the procedure, reliable identification of the tachycardia circuit and the appropriate ablation site and creation of an effective tissue-altering lesion. This article will focus on the identification of the intra-atrial tachycardia circuit by looking at the most recent developments in mapping, and will also present the two main patient populations that are encountered in the electrophysiology (EP) lab: patients after the Fontan procedure and patients with D-transposition of the great arteries after an atrial switch procedure (Mustard or Senning procedure).
The Use of Mapping Systems
In contrast to a ‘healthy’ atrium with defined anatomical structures, scarring, pressure overload or hypoxia-related changes can lead to the development of complex intra-atrial re-entrant tachycardias (IARTs) in patients after congenital heart disease surgery. Often, conventional mapping fails to depict these re-entrant circuits.8
In most cases IART is dependent on areas of surgical scarring, which function as lines of conduction slowing or block. Therefore, it is important to identify areas of absent or low voltage (<0.03mV), which are catalogued as incisional scars or patches (e.g. coloured grey on CARTO® maps). Other important insights into tachycardia mechanisms are given by ‘double potentials’ – two discrete electrocardiograms separated by at least 20msec. For example, a line of double potentials situated on the free wall of the right atrium (RA) may correlate with an atriotomy scar (a line of conduction block with asynchronous activation on either side). ‘Fractionated’ potentials show continuous low amplitude (<0.1mV) with more than two separate positive or negative deflections. These may also correspond to areas of slow or blocked conduction, or may indicate the site of origin of a focal atrial tachyardia.9
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- Weipert J, Noebauer C, Schreiber C, et al., Occurrence and management of atrial arrhythmia after long-term Fontan circulation, J Thorac Cardiovasc Surg, 2004;127(2):457–64.
- Kaemmerer H, Fratz S, Bauer U, et al., Emergency hospital admissions and three-year survival of adults with and without cardiovascular surgery for congenital cardiac disease, J Thorac Cardiovasc Surg, 2003;126(4):1048–52.
- Posner P, Prestwich KN, Buss DD, Cardiac maturation in an hypoxic milieu: implications for arrhythmias in hypoxemic defects, Pediatr Res, 1985;19(1):64–6.
- Ishii Y, Nitta T, Sakamoto S, et al., Incisional atrial reentrant tachycardia: experimental study on the conduction property through the isthmus, J Thorac Cardiovasc Surg, 2003;126(1): 254–62.
- Love BA, Collins KK, Walsh EP, Triedman JK, Electroanatomic characterization of conduction barriers in sinus/atrially paced rhythm and association with intra-atrial reentrant tachycardia circuits following congenital heart disease surgery, J Cardiovasc Electrophysiol, 2001;12(1):17–25.
- Saul JP, Walsh EP, Triedman JK, Mechanisms and therapy of complex arrhythmias in pediatric patients, J Cardiovasc Electrophysiol, 1995;6(12):1129–48.
- Dorostkar PC, Cheng J, Scheinman MM, Electroanatomical mapping and ablation of the substrate supporting intraatrial reentrant tachycardia after palliation for complex congenital heart disease, Pacing Clin Electrophysiol, 1998;21(9):1810–19.
- de Groot NM, Zeppenfeld K, Wijffels MC, et al., Ablation of focal atrial arrhythmia in patients with congenital heart defects after surgery: role of circumscribed areas with heterogeneous conduction, Heart Rhythm, 2006;3(5):526–35.
- Pflaumer A, Hessling G, Luik A, et al., Remote Magnetic Catheter Mapping and Ablation of Permanent Junctional Reciprocating Tachycardia in a Seven-Year-Old Child, J Cardiovasc Electrophysiol, 2007.
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- Pflaumer A, Hessling G, Luik A, et al., Mapping von supraventrikulären Tachykardien mit Magnetnavigation– erste Erfahrungen bei Kindern und Patienten mit komplexen Herzfehlern, Clin Res Cardiol, 2007;96(Suppl. 1).
- Triedman JK, Bergau DM, Saul JP, et al., Efficacy of radiofrequency ablation for control of intraatrial reentrant tachycardia in patients with congenital heart disease, J Am Coll Cardiol, 1997;30(4):1032–8.
- Lukac P, Pedersen AK, Mortensen PT, et al., Ablation of atrial tachycardia after surgery for congenital and acquired heart disease using an electroanatomic mapping system: Which circuits to expect in which substrate?, Heart Rhythm, 2005;2(1):64–72.
- Abrams D, Schilling R, Mechanism and mapping of atrial arrhythmia in the modified Fontan circulation, Heart Rhythm, 2005;2(10):1138–44.
- Deisenhofer I, Estner H, Pflaumer A, Zrenner B, Atypical access to typical atrial flutter, Heart Rhythm, 2005;2(1):93–6.
- Triedman JK, DeLucca JM, Alexander ME, et al., Prospective trial of electroanatomically guided, irrigated catheter ablation of atrial tachycardia in patients with congenital heart disease, Heart Rhythm, 2005;2(7):700–5.
- Pflaumer A, Haimerl M, Zrenner B, et al., Focal Atrial Tachycardia in Patients after Fontan Operation: an Underestimated Problem, Europace, 2006;246:5.
- Zrenner B, Dong J, Schreieck J, et al., Delineation of intra-atrial reentrant tachycardia circuits after mustard operation for transposition of the great arteries using biatrial electroanatomic mapping and entrainment mapping, J Cardiovasc Electrophysiol, 2003;14(12):1302–10.
- Dong J, Zrenner B, Schreieck J, Schmitt C, Necessity for biatrial ablation to achieve bidirectional cavotricuspid isthmus conduction block in a patient following senning operation, J Cardiovasc Electrophysiol, 2004;15(8):945–9.
- 15 January 2009




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