Korean Circ J.  2015 Jul;45(4):344-347. 10.4070/kcj.2015.45.4.344.

Transvascular Implantation of an Implantable Cardioverter-Defibrillator in a Patient Who has Undergone One-and-a-Half Ventricle Repair

• Yang PS
• Park JW
• Lee YJ
• Kim DJ
• You SC
• Park DH
• Uhm JS
• Kim NK

• KMID: 1964109
• DOI: http://doi.org/10.4070/kcj.2015.45.4.344

Abstract

Implantable cardioverter-defibrillator (ICD) therapy is acknowledged as a valid treatment method for the effective prevention of sudden cardiac death, which is a major cause of mortality in adult congenital heart disease patients. But ICD implantation by the conventional transvascular approach is not always possible in patients who have undergone palliative surgery due to congenital and structural heart disease. Here, we report a case in which an ICD was transvascularly implanted in an arrhythmogenic right ventricular cardiomyopathy patient who had undergone a one-and-a-half ventricle repair.

Keyword

Implantable cardioverter-defibrillator; Heart defects, congenital; Arrhythmogenic right ventricular dysplasia; Bidirectional cavopulmonary shunt

MeSH Terms

Adult
Arrhythmogenic Right Ventricular Dysplasia
Death, Sudden, Cardiac
Defibrillators, Implantable*
Fontan Procedure
Heart Defects, Congenital
Heart Diseases
Humans
Mortality
Palliative Care

Figure

• 

  Fig. 1 Patient's ECG when he visited the emergency department with palpitations and pre-syncope. ECG showed monomorphic ventricular tachycardia at the rate of 225 bpm. ECG: electrocardiogram.

• 

  Fig. 2 A schematic picture describing the one-and-a-half ventricle repair and chest X-ray image of the implanted ICD. A: a schematic picture describing the one-and-a-half ventricle repair is drawn on the patient's chest X-ray. Gray arrows represent the blood flow. B: the chest X-ray shows that the ICD is placed in the right pectoral area in the RV via the cavopulmonary shunt. ICD: implantable cardioverter-defibrillator, PA: pulmonary artery, RV: right ventricle, SVC: superior vena cava.

• 

  Fig. 3 Heart magnetic resonance images. Short axis cine image with SSFP sequence (A), four chamber cine image with SSFP sequence (B), delayed enhanced image (C). Severe right ventricular enlargement with a dyskinetic segment and right ventricular wall thinning is noted (arrowheads). In the contrast study, extensive delayed hyperenhancement which indicates fi brous displacement is also shown (arrow). These fi ndings of the heart MRI meet the criteria for a diagnosis of ARVC. ARVC: arrhythmogenic right ventricular cardiomyopathy, MRI: magnetic resonance imaging, SSFP: steady state free precession.

Reference

1. Winkle RA, Mead RH, Ruder MA, et al. Long-term outcome with the automatic implantable cardioverter-defibrillator. J Am Coll Cardiol. 1989; 13:1353–1361.

2. Koyak Z, Harris L, de Groot JR, et al. Sudden cardiac death in adult congenital heart disease. Circulation. 2012; 126:1944–1954.

3. Mondésert B, Khairy P. Implantable cardioverter-defibrillators in congenital heart disease. Curr Opin Cardiol. 2014; 29:45–52.

4. Cannon BC, Friedman RA, Fenrich AL, Fraser CD, McKenzie ED, Kertesz NJ. Innovative techniques for placement of implantable cardioverter-defibrillator leads in patients with limited venous access to the heart. Pacing Clin Electrophysiol. 2006; 29:181–187.

5. Uyeda T, Inoue K, Sato J, et al. Outcome of implantable cardioverter defibrillator therapy for congenital heart disease. Pediatr Int. 2012; 54:379–382.

6. Nery PB, Green MS, Khairy P, Alhebaishi Y, Hendry P, Birnie DH. Implantable cardioverter-defibrillator insertion in congenital heart disease without transvenous access to the heart. Can J Cardiol. 2013; 29:254.

7. Khanna AD, Warnes CA, Phillips SD, Lin G, Brady PA. Single-center experience with implantable cardioverter-defibrillators in adults with complex congenital heart disease. Am J Cardiol. 2011; 108:729–734.

8. Akerström F, Arias MA, Pachón M, Puchol A, Jiménez-López J. Subcutaneous implantable defibrillator: state-of-the art 2013. World J Cardiol. 2013; 5:347–354.

9. Lambiase PD, Barr C, Theuns DA, et al. Worldwide experience with a totally subcutaneous implantable defibrillator: early results from the EFFORTLESS S-ICD Registry. Eur Heart J. 2014; 35:1657–1665.

10. Kreutzer C, Mayorquim RC, Kreutzer GO, et al. Experience with one and a half ventricle repair. J Thorac Cardiovasc Surg. 1999; 117:662–668.