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

Abstract

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 dextrotransposition 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.

Congenitally Corrected Transposition of the Great Arteries in the Adult

“…Mechanical support in centers with programs that provide such support should be considered as a bridge to transplant or destination therapy (depending on the patient’s age and social situation) for whom heart failure is refractory to medical and any other adjunct therapy, like resynchronization therapy. A recent review focusing on patients with failing systemic RV draw attention for the potential benefits of this technique, but reinforced the need for multicenter investigations in pediatric and adult cohorts with short and long-term assessment [ 67 ] . Any decisions regarding surgical intervention should take the individual’s anatomy and clinical status into account so that patients are offered the options that offers the least risk and has the highest likelihood of improving the patient’s current clinical status and quality of life.…”

Double outlet right ventricle in adults: Anatomic variability, surgical treatment, and late postoperative complications

“…[68][69][70] Besides, there is a growing interest in cardiac resynchronization therapy in patients with failing systemic RV, signifying the possibility to restore the mechanical synchronization of contractility in both ventricles of the heart. 71 While the duration and fragmentation of QRS complex is an established risk factor for sudden cardiac death in patients with tetralogy of Fallot, in the study by Shen et al…”

“…However, some authors argue for the existence of significant impact of RBBB on both right and left ventricular systolic function 68–70 . Besides, there is a growing interest in cardiac resynchronization therapy in patients with failing systemic RV, signifying the possibility to restore the mechanical synchronization of contractility in both ventricles of the heart 71 . While the duration and fragmentation of QRS complex is an established risk factor for sudden cardiac death in patients with tetralogy of Fallot, in the study by Shen et al the presence of either RBBB or left bundle branch block in 89 patients with operated DORV was not associated with increased risk of sudden cardiac death 62 …”

Arrhythmias in adults with congenital heart disease and heart failure

“…These particularities and unique considerations limit the extrapolation of data from trials in patients with acquired heart disease. Nevertheless, encouraging results have been reported from observational studies in patients with heterogeneous forms of CHD, [36][37][38][39][40][41][42][43][44] including systemic right ventricular dysfunction. [45][46][47] In the CHD population that deviates from conventional criteria, the most established indication for CRT is the patient with CCTGA who requires ventricular pacing (Figure 3).…”