Patients who have undergone repair of congenital heart disease are at risk of atrial and ventricular tachyarrhythmias and sudden cardiac death (SCD), both because of their arrhythmia substrate and their altered hemodynamic response to it. While patients with even complex cardiac defects now have a realistic chance to survive into adulthood, cardiac arrhythmias are a very common source of morbidity and mortality in this patient group ( Table 18.1 ).

TABLE 18.1
Typical Arrhythmia in Selected Types of Adult Congenital Heart Disease
Modified from Walsh EP. Interventional electrophysiology in patients with congenital heart disease. Circulation . 2007;115:3224-3234.
Type of ACHD IART AP-Mediated Arrhythmia Atrial Fibrillation VT SCD
Atrial septal defect +++ +
Ventricular septal defect +
Ebstein anomaly of the tricuspid valve + (RA isthmus-dependent flutter) +++ ++
Tetralogy of Fallot ++ + +++ +
Transposition of great arteries (post Mustard or Senning) +++ + +
Single ventricle (Fontan) +++ ++
Total cavopulmonary connection ++ +
ACHD, Adult congenital heart disease; AP, accessory pathway; IART, intraatrial reentry tachycardia; SCD, sudden cardiac death; RA, right atrial; VT, ventricular tachyarrhythmia.

Treating arrhythmias in this patient cohort is challenging for many reasons: the need to understand the patient’s anatomy at baseline and after surgical modification, further change of the anatomic substrate because of myocardial hypertrophy and fibrosis caused by chronic hemodynamic overload, and identification of the underlying tachycardia mechanism(s). Even if all these issues are taken into consideration, delivery of therapy (eg, catheter ablation or lead placement) can be challenging because of the sheer size or location of the target area.

Development of sophisticated mapping systems in recent years has allowed us to address catheter ablation with reasonable chances of success; devices to treat bradycardia and prevent SCD have improved equally, but still carry a significant risk of failure.

Invasive Electrophysiology in Adult Congenital Heart Disease Patients With Atrial Arrhythmias

The most common arrhythmia mechanism in patients with adult congenital heart disease (ACHD) involves a macroreentrant circuit within the atria (intraatrial reentry tachycardia [IART]). These IARTs occur most commonly around anatomic obstacles such as patches or suture lines (eg, at the bypass cannulation sites or atriotomies). Knowing the type of surgical procedure performed, including the presence of artificial material (such as patches or baffles), helps in narrowing down the potential reentrant circuits significantly. However, focal tachycardias are also not uncommon and can be difficult to map and understand, especially in patients with large scar areas. Detailed mapping and confirmation of the three-dimensional (3D) mapping data by conventional electrophysiology (EP) pacing maneuvers are key for a successful ablation procedure.

Understanding the Anatomy

In recent years, the accuracy and availability of 3D imaging modalities such as computed tomography (CT), cardiac magnetic resonance (CMR) imaging, or echocardiography have improved dramatically. Especially in the presence of ACHD, a 3D reconstruction of the individual anatomy of a given patient is very helpful ( Fig. 18.1 ). Familiarization with the underlying anatomy helps facilitate any electrophysiologic intervention and allows planning of optimal access routes (eg, retrograde through a hemiazygos continuation). Direct 3D imaging of scar tissue, for example, by late enhancement in CMR, might prove as valuable in ACHD patients as recently demonstrated in patients undergoing atrial fibrillation ablation. Knowing the dimensions of the target chamber helps in choosing the proper tools (eg, large curves or long guiding sheaths). In some instances, access to the target chamber might be obstructed by artificial valves, which might necessitate transseptal or transbaffle puncture.

Figure 18.1, A, Noncontrast three-dimensional (3D) cardiac magnetic resonance imaging of a patient with transposition of the great arteries and atrial switch operation (Mustard). B, 3D reconstruction of the same image data to allow detailed procedure planning (Polaris, Biosense Webster).

Use of Three-Dimensional Mapping Systems for Catheter Ablation in Adult Congenital Heart Disease Patients

With the advent of 3D EP mapping systems, catheter ablation of IART experienced a “quantum leap.” These systems helped display the cardiac chambers in three dimensions, greatly facilitated understanding of the underlying mechanisms, and thereby reduced the total fluoroscopy exposure. Success rates reported for ACHD arrhythmias increased accordingly for acute and chronic results.

Integration of the pre-acquired 3D images is now standard for all 3D mapping systems, allowing the electrophysiologic information to be superimposed on the 3D contour ( Fig. 18.2 ).

Figure 18.2, Example of magnetically remote-controlled mapping of the pulmonary venous atrium (PVA) in a patient after Mustard operation. Top, Fused cardiac magnetic resonance 3D reconstruction on the fluoroscopy reference screens in both right anterior oblique (RAO) and left anterior oblique (LAO) projections. Bottom, The same situation with the magnetic catheter (Magn. ABL) advanced retrograde via the aorta (Ao) through the right ventricle (RV) into the lateral inferior pulmonary vein of the PVA.

Sequential Versus Simultaneous Mapping

In the last 5 years, simultaneous mapping systems have been introduced to the invasive EP arena (contact mapping using multielectrode baskets or noninvasive body surface mapping combined with 3D imaging). Data on patients with ACHD currently exist for noninvasive body surface mapping combined with 3D imaging. This system simultaneously records from 252 surface ECG electrodes and displays the electrical information of each cardiac activation on a 3D epicardial reconstruction of the biatrial or biventricular chambers ( Fig. 18.3 ). This allows mapping of multiple arrhythmias or even very rare arrhythmias (eg, ventricular ectopy triggering ventricular fibrillation) while the patient is still on the ward. Mapping can be performed for several hours, and provocation, such as physical exercise on a stationary bike or with various common stimulants (food, social interaction, pharmacologic, etc.), is carried out on the ward rather than in the catheterization lab.

Figure 18.3, Example of a simultaneous mapping system that reconstructs the surface ECGs of 252 electrodes (left upper panel) on the atrial or ventricular epicardial three-dimensional (3D) reconstructions from a noncontrast computed tomography (CT) scan (right upper panel) . Bottom panels display the 3D mapping information with and without antegrade conduction across an accessory pathway (AP) in posterolateral orientation of the tricuspid annulus (TA) in a patient with Ebstein anomaly. Ao, Aorta; MA, mitral annulus.

In the field of sequential mapping systems, multielectrode mapping has been introduced to shorten the time required for mapping of a given arrhythmia. However, it is critical for these systems that the arrhythmia is relatively stable with little cycle length variation. Also, because direct contact is required, the risk of mechanical alteration or termination is higher. Fig. 18.4 shows an example of the Rhythmia system (Boston Scientific, Marlborough, Massachusetts), which collects a large number of points with a dedicated multielectrode basket catheter. Comparison of neighboring points and various other stability criteria (including cycle length stability) allows rapid mapping of several thousand points on a 3D reconstruction. No data are yet available using this technology in a patient cohort with ACHD.

Figure 18.4, Example of a patient with juxtapositioned atrial appendages and atrial tachycardia. Top panel, three-dimensional (3D) reconstruction from contrast computed tomography (CT) scan depicting the juxtapositioned right (RAA) and left atrial appendage (LAA). Bottom left panel, full reconstruction of the atrial activation sequence using the sequential multielectrode mapping system Rhythmia (Boston Scientific). The reentry circuit rotates around a centrally located scar (gray area) with the critical isthmus between the scar and the superior vena cava (SVC). Right bottom panel, same as left but displayed in right anterior oblique (RAO) projection to display large scar area (in gray) in the free wall of the massively dilated right atrium. Red catheter displayed served as the timing reference during the mapping process with the Orion basket catheter. LA, Left atrium; MA, mitral annulus; RA, right atrium; TA, tricuspid annulus.

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