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When to implant an ICD in systemic right
ventricle ?
Département de rythmologie
et de stimulation cardiaque
Nicolas Combes
[e-mail]
Pôle de cardiologie pédiatrique
et congénitale

Risk of sudden death
ICD related
complications

Factors influencing sudden death
Systemic RV
Ejection Fraction
Supraventricular arrhythmias
Ventricular scars
Complex ventricular arrhythmias
Functional class (NYHA)
Electrophysiological markers
- QRS duration
- QRS dispersion
- Programmed ventricular stimulation

A wide spectrum of cardiac abnormalities and surgery
Congenitally corrected transposition of great arteries
- native without surgery
- ventricular surgery for VSD
- conduit between LV and pulmonary artery
-double switch…
Complete transposition of great arteries after atrial switch (Mustard and
Senning) with possible ventricular sugery
Single right ventricle physiology +/- Fontan circulation or aortopulmonary shunt
- Hypoplastic left heart syndrome
- Mitral atresia
- double inlet right ventricle
- unbalanced common AV canal defect …
Not the same sudden death risk

Koyak Z et al. Circulation 2012;126:1944
Database with 25790 adults (Netherlands, Belgium and Canada) with CHD
1189 death (5%), 213 sudden death (171 arrythmic)
X
ccTGA (n=12): 75% with associated defect and surgery (VSD, pulmonary stenosis)
dTGA (n=18) : 83% Mustard or Senning repair