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9.2 Patients With Adult Congenital Heart Disease

See Figure 22 for the algorithm for acute treatment of non– pre-excited SVT in adult congenital heart disease (ACHD) patients; and Figure 23 for the algorithm for ongoing management of non–pre-excited SVT in ACHD patients.

9.2.1 Clinical Features

SVT is observed in 10% to 20% of ACHD patients, and is associated with a significantly increased risk of heart failure, stroke, and SCD.440–444 The most common mechanism of SVT in ACHD patients is macroreentrant AT (also called flutter), which accounts for at least 75% of SVT and frequently involves the CTI. Focal AT, AVNRT, and accessory pathway–mediated tachycardia each account for less than about 8% of SVT, whereas the incidence of AF is about 10% and increases with age.133,445–449 AT occurs in 20% to 45% of adults with Ebstein anomaly, single-ventricle/ Fontan procedures, tetralogy of Fallot, transposition of the great arteries, and atrial septal defects.449–451

The management of SVT in ACHD patients is influenced by the underlying cardiac anatomy and surgical repair, the current hemodynamic sequelae of the anatomy and repairs, and mechanism of SVT. The ventricular rate during SVT may be slowed because of variable AV conduction, which can result in a delay in recognizing tachycardia and the development of congestive failure. Recognition of severe forms of congenital heart disease, including unrepaired or palliated defects,

Table 8.  Success and Complication Rates for Ablation of SVT*

Arrhythmia Acute Success Recurrence Rate Major Complications References Common SVTs

AVNRT 96%–97%102,103 5%103 •  Overall 3%102 102,103 •  PPM 0.7%102

•  Death 0%102

AVRT/accessory pathway 93%102,103 8%103 •  Overall 2.8%102 102,103 •  PPM 0.3%102

•  Death 0.1%102 •  Tamponade 0.4%102

CTI-dependent atrial flutter 97%102 10.6% atrial flutter,121 33% atrial fibrillation121 •  Overall 0.5%102 102,103,121 •  PPM 0.2%102

•  Pericardial effusion 0.3%102

Less common SVTs

Focal AT 80%–100% 4%–27% <1%–2% 122–129 JT 82%–85% 0–18% 0–18% CHB (overall complications N/A) 130–132 Non–CTI-dependent atrial flutter 73%–100% 7%–53% 0–7% 122,133–140 *Data in this table are derived from multiple observational studies and registries, and as such may not always reflect current practice. AT indicates atrial tachycardia; AVNRT, atrioventricular nodal reentrant tachycardia; AVRT, atrioventricular reentrant tachycardia; CHB, complete heart block; CTI, cavotricuspid isthmus; JT, junctional tachycardia; N/A, not available; PPM, permanent pacemaker; and SVT, supraventricular tachycardia.

Figure 20. Surface electrocardiographic recording from leads VI, II, and V5 in a patient with junctional tachycardia. The upper panel shows sinus rhythm. The lower panel shows tachycardia onset with the characteristic finding of dissociation of the QRS and P waves (narrow arrows on P waves). The large arrow signifies continuous recording. Reproduced with permission from Blomström-Lundqvist et al.11 Figure 21. Ongoing management of junctional tachycardia. Colors correspond to Class of Recommendation in Table 1; drugs listed alphabetically. SHD indicates structural heart disease (including ischemic heart disease).

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