The systemic right ventricle (SRV), defined as the morphological right ventricle supporting the systemic circulation, is relatively common in congenital heart disease (CHD). Our review aimed at examining the current evidence, knowledge gaps and technical considerations regarding implantable cardiac electronic device therapy in patients with SRV. The risk of sinus node dysfunction (SND) after atrial switch repair and/or complete heart block in congenitally corrected transposition of great arteries requiring permanent pacing increases with age. Similar to acquired heart disease, indication for pacing includes symptomatic bradycardia, SND and high degree atrioventricular nodal block. Right ventricular dysfunction and heart failure also represent important complications in SRV patients. Cardiac resynchronisation therapy (CRT) has been proposed to improve systolic function in SRV patients, although indications for CRT are not well defined and its potential benefit remains uncertain. Amongst adult CHD, patients with SRV are at the highest risk for sudden cardiac death (SCD). Nevertheless, risk stratification for SCD is scarce in this cohort and implantable cardioverter-defibrillator indication is currently limited to secondary prevention. Vascular access and the incidence of device-related complications, such as infections, inappropriate shocks and device system failure, represent additional challenges to implantable cardiac electronic device therapy in patients with SRV. A multidisciplinary approach with tertiary expertise and future collaborative research are all paramount to further the care for this challenging nonetheless ever increasing cohort of patients.
- congenital heart disease
- implanted cardiac defibrillators
- complex congenital heart disease
- transposition of the great arteries
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Contributors RB and TW made substantial contribution in writing the article and revising it critically for important intellectual content. MB, SG, RP, GPD and MAG made substantial contributions. RB is responsible for the overall content as guarantor. All authors gave final approval of the version to be submitted and any revised version.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting, or dissemination plans of this research.
Patient consent for publication Not required.
Provenance and peer review Not commissioned; externally peer reviewed.
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