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ICD therapy for primary prevention of sudden cardiac death after Mustard repair for d-transposition of the great arteries

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Abstract

Background

Years and decades after the Mustard atrial switch repair for d-Transposition of the great arteries, there is a subset of patients at increased risk for sudden cardiac death due to ventricular tachyarrhythmias. To date, little is known about indications, efficacy and benefit of internal cardioverter defibrillator (ICD) therapy indication in these patients.

Patients/methods

To characterize the Mustard patients already treated with an ICD, we conducted a single center case control study (n = 41). The charts of all patients after Mustard procedure at our institution were systematically reviewed for history, echocardiographic findings, arrhythmias, hemodynamics and medication as well as ICD discharges and complications.

Results

Significant differences between ICD (n = 12) and non-ICD patients (n = 29) were found regarding the stage of heart failure, need for heart failure medication, QRS duration and left ventricular diameter and performance. Inappropriate ICD discharges due to rapidly conducted atrial reentrant tachycardia and sensing failure were frequently observed. In 17 % of our Mustard patients with an ICD, infection of the implantation site required surgical revision. All ICD patients were of male gender.

Conclusion

Patients after ICD implantation represent a subgroup among our Mustard patients with a more advanced state of cardiac disease. Not only systemic right ventricular diameter and performance but also subpulmonary left ventricular performance was an important factor predicting the long-term course of these patients. Prevention of inappropriate discharges requires decent device programming, close monitoring of lead integrity and treatment of atrial tachycardias.

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Abbreviations

ART:

Atrial reentrant tachycardia

EPS:

Electrophysiological study

ICD:

Internal cardioverter defibrillator

IQR:

Interquartile range

LV:

Left ventricle

PVC:

Premature ventricular contraction

RV:

Right ventricle

SCD:

Sudden cardiac death

TGA:

Transposition of the great arteries

VSD:

Ventricular septal defect

VT:

Ventricular tachycardia

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Conflict of interest

The authors have no conflicts of interest to disclose.

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Authors and Affiliations

  1. Department of Pediatric Cardiology and Intensive Care Medicine, University Hospital, Georg-August-University, Robert-Koch-Str 40, 37075, Göttingen, Germany

    David Backhoff, Matthias Müller, Thomas Paul & Ulrich Krause

  2. Department of Thoracic and Cardiovascular Surgery, University Hospital, Georg-August-University, Göttingen, Germany

    Wolfgang Ruschewski

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  1. David Backhoff
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  2. Matthias Müller
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  5. Ulrich Krause
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Correspondence to Ulrich Krause.

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Backhoff, D., Müller, M., Ruschewski, W. et al. ICD therapy for primary prevention of sudden cardiac death after Mustard repair for d-transposition of the great arteries. Clin Res Cardiol 103, 894–901 (2014). https://doi.org/10.1007/s00392-014-0727-x

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