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Review
. 2023 Jun 5:13:100463.
doi: 10.1016/j.ijcchd.2023.100463. eCollection 2023 Sep.

Ventricular arrhythmia in congenital heart diseases with a systemic right ventricle

Magalie Ladouceur  1 , Victor Waldmann  1 , Stefano Bartoletti  2 , Marie-A Chaix  2 , Paul Khairy  2
Affiliations
Review

Ventricular arrhythmia in congenital heart diseases with a systemic right ventricle

Magalie Ladouceur et al. Int J Cardiol Congenit Heart Dis. .

Erratum in

Abstract

Congenital heart disease (CHD) often involves the systemic right ventricle (SRV), which is the morphological right ventricle that supports systemic circulation. SRV patients are at a higher risk of sudden cardiac death (SCD) than other adult CHD patients and continues to be a significant cause of death in this aging population. However, the pathophysiology of ventricular arrhythmias in SRV is still not fully understood, and there may be differences between subtypes of CHD. Although these events are rare, predicting them is challenging. This review discusses contemporary strategies for assessing and preventing the risk of ventricular arrhythmias in SRV patients. Several risk factors have been identified to be associated with ventricular arrhythmias in patients with SRV. A recent risk stratification model combines independently associated factors into a risk score, and subpulmonary left ventricle dysfunction is emerging as a critical factor in risk assessment. Cardiac magnetic resonance imaging, biomarkers, and genetic data may refine the ability to predict ventricular arrhythmias in SRV. However, the question of whether implantable cardioverter-defibrillators (ICDs) should be used as a preventive measure in this cohort remains unanswered. Multicenter studies are needed to evaluate risk models and ICD use in this aging population. Given that ICDs have drawbacks, such as a high rate of inappropriate shocks and late lead-related complications, shared clinical decision-making is crucial when considering their use. The review emphasizes the need for further research in this area to improve the identification of patients at risk of clinical ventricular arrhythmias and to develop effective prevention strategies.

Keywords: Implantable cardioverter-defibrillator; Sudden cardiac death; Systemic right ventricle; Transposition of the great arteries; Ventricular arrhythmia.

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Conflict of interest statement

The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

Figures

Fig. 1
Fig. 1
Patient with congenitally corrected transposition of the great arteries and biventricular implanted cardioverter defibrillator who received a shock to treat ventricular fibrillation following an R-on-T phenomenon.
Fig. 2
Fig. 2
Late gadolinium lesions in patients with D-TGA. LGE distribution was mainly in the basal segments of the sRV free wall (Panel B) and correlation with macroscopic fibrosis in heart explanted (Panel C).
Fig. 3
Fig. 3
Potential triggers and substrates for SCD in D-TGA and L-TGA (congenitally corrected TGA). Strengths of associations are semi-quantitively estimated by number of asterisks from absent/weak (*) to moderate (**), strong (***), and very strong (****). Reproduced with permission from Khairy P. Eur Heart J 2022; 3:2695–7 [34].
See this image and copyright information in PMC

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