Cardiovascular Journal of Africa: Vol 25 No 6 (November/December 2014)

CARDIOVASCULAR JOURNAL OF AFRICA • Volume 25, No 6, November/December 2014

AFRICA

Case Report

Clinical ventricular tachycardia and surgical epicardial

ICD implantation in a patient with a Fontan operation for

double-inlet left ventricle

Aysen Agacdiken Agir, Umut Celikyurt, Kurtulus Karauzum, Irem Yilmaz, Ersan Ozbudak, Serdar Bozyel,

Muhip Kanko, Ahmet Vural, Dilek Ural

Summary

The Fontan operation is the primary surgical technique used

for palliation of patients with single-ventricle physiology.

Arrhythmias are frequently observed and associated with

morbidity and mortality in Fontan patients. The frequency

of arrhythmias after the Fontan procedure increases over time

and it was reported to reach 50% in a 20-year follow up. Atrial

tachyarrhythmias, especially atrial tachycardia and sinus

bradycardia, are most frequently observed in these patients.

Ventricular arrhythmias are rarely observed.

Generally, medical therapy, catheter ablation, pacemaker

or implantable cardioverter defibrillator (ICD) implanta-

tion are options in the treatment of these arrhythmias. It

may be difficult to implant either a pacemaker or an ICD in

patients on whom the Fontan procedure has been performed.

In conditions where access to the right ventricle is from the

venous system, it is anatomically impossible. Where there

is no functional right ventricle, device implantation can be

performed with alternative methods other than the conven-

tional transvenous approach.

In this report, we discuss a middle-aged woman with a

Fontan operation performed 14 years earlier, who presented

with ventricular tachycardia (VT) and in whom an epicar-

dial ICD was implanted. The literature on this issue is also

reviewed.

Keywords:

Fontan operation, ventricular arrhythmia, implant-

able cardioverter defibrillator, epicardial

Submitted 11/2/14, accepted 18/9/14

Cardiovasc J Afr

2014;

: e6–e10

www.cvja.co.za

DOI: 10.5830/CVJA-2014-057

Double-inlet ventricle falls under the umbrella of univentricular

atrio-ventricular (AV) connections. These hearts are defined as

having more than 50% of each AV connection connected to a

dominant ventricle. In about 75% of patients, the dominant

(functional) ventricle is a left ventricle, separated from the

right ventricle by a rudimentary interventricular septum.

These patients usually undergo Fontan surgery with a surgical

algorithm such as in those with tricuspid atresia.

The frequency of arrhythmias after the Fontan procedure

increases over time and it was reported at 50% in a 20-year follow

up.

2,3

Early postoperative arrhythmias can result from sinus node

or sinus node artery trauma. In the late period, arrhythmias can

result from atrial dilatation and distension and surgical scars.

Atrial tachyarrhythmias, especially atrial tachycardia and sinus

bradycardia, are most frequently observed in these patients.

Ventricular arrhythmias are rarely observed. However, sudden

cardiac death is the most common cause of mortality in adult

congenital heart disease. Generally, medical therapy, catheter

ablation, pacemaker or implantable cardioverter defibrillator

(ICD) implantation are the options in the treatment of these

arrhythmias.

In this article we report on a middle-aged woman with a

Fontan operation 14 years earlier, presenting with ventricular

tachycardia (VT) and in whom an epicardial ICD was implanted.

The literature on this issue is reviewed.

Case report

A 43-year-old woman was admitted to the emergency department

with palpitations accompanied by pre-syncope. There was no

history of syncope. She had had a Fontan operation due to

a double-inlet left ventricle 14 years earlier. Revision of the

previous Fontan connections and an epicardial VVIR pacemaker

implantation were done due to bradycardia nine years earlier.

On physical examination, her vital signs showed a body

temperature of 36.7°C, a pulse of 190 beats/min, and blood

pressure of 80/50 mmHg. Cardiac auscultation revealed beat-

to-beat changes and softening of S1, mildly loud S2 and

Department of Cardiology, Kocaeli University Medical

Faculty, Kocaeli, Turkey

Aysen Agacdiken Agir, MD,

aagacdiken@yahoo.com

Umut Celikyurt, MD

Kurtulus Karauzum, MD

Irem Yilmaz, MD

Serdar Bozyel, MD

Ahmet Vural, MD

Dilek Ural, MD

Department of Cardiovascular Surgery, Kocaeli University

Medical Faculty, Kocaeli, Turkey

Ersan Ozbudak, MD

Muhip Kanko, MD