Cardiac resynchronization therapy for the failing systemic right ventricle: A systematic review

Patients with a systemic right ventricle (SRV) are at high risk for development of heart failure early in life. An SRV is encountered in patients with congenitally corrected transposition of the great arteries (CCTGA) or dextro-transposition of the great arteries (DTGA) with previous atrial switch repair (Mustard or Senning procedure). Progressive heart failure is one of the leading cause of mortality in these patients. Therefore, cardiac resynchronization therapy (CRT) has gained increasing momentum for use in this challenging congenital heart disease (CHD) population. However, current guidelines differ in recommendations for CRT in patients with an SRV as evidence supporting CRT has thus far only been described in case reports and retrospectively in relatively small study populations. In fact, the European Society of Cardiology Guideline for the management of grown-up congenital heart disease consider CRT to be 'experimental' in this population. This systematic review critically summarizes current literature on CRT in SRV patients and provides future perspectives for further research in this challenging and growing CHD population.