Impact and Modifiers of Ventricular Pacing in Patients With Single Ventricle Circulation

Henry Chubb, Anica Bulic, Douglas Mah, Jeremy P. Moore, Jan Janousek, Jennifer Fumanelli, S. Yukiko Asaki, Andreas Pflaumer, Allison C. Hill, Carolina Escudero, Sit Yee Kwok, Jasveer Mangat, Luis A. Ochoa Nunez, Seshadri Balaji, Eric Rosenthal, William Regan, Michaela Horndasch, Hiroko Asakai, Ronn Tanel, Richard J. CzosekMing Lon Young, David J. Bradley, Thomas Paul, Peter Fischbach, Lindsey Malloy-Walton, Doff B. McElhinney, Anne M. Dubin

Research output: Contribution to journalArticlepeer-review

3 Scopus citations


Background: Palliation of the single ventricle (SV) circulation is associated with a burden of lifelong complications. Previous studies have identified that the need for a permanent ventricular pacing system (PPMv) may be associated with additional adverse long-term outcomes. Objectives: The goal of this study was to quantify the attributable risk of PPMv in patients with SV, and to identify modifiable risk factors. Methods: This international study was sponsored by the Pediatric and Congenital Electrophysiology Society. Centers contributed baseline and longitudinal data for functionally SV patients with PPMv. Enrollment was at implantation. Controls were matched 1:1 to PPMv subjects by ventricular morphology and sex, identified within center, and enrolled at matched age. Primary outcome was transplantation or death. Results: In total, 236 PPMv subjects and 213 matched controls were identified (22 centers, 9 countries). Median age at enrollment was 5.3 years (quartiles: 1.5-13.2 years), follow-up 6.9 years (3.4-11.6 years). Median percent ventricular pacing (Vp) was 90.8% (25th-75th percentile: 4.3%-100%) in the PPMv cohort. Across 213 matched pairs, multivariable HR for death/transplant associated with PPMv was 3.8 (95% CI 1.9-7.6; P < 0.001). Within the PPMv population, higher Vp (HR: 1.009 per %; P = 0.009), higher QRS z-score (HR: 1.19; P = 0.009) and nonapical lead position (HR: 2.17; P = 0.042) were all associated with death/transplantation. Conclusions: PPMv in patients with SV is associated with increased risk of heart transplantation and death, despite controlling for increased associated morbidity of the PPMv cohort. Increased Vp, higher QRS z-score, and nonapical ventricular lead position are all associated with higher risk of adverse outcome and may be modifiable risk factors.

Original languageEnglish (US)
Pages (from-to)902-914
Number of pages13
JournalJournal of the American College of Cardiology
Issue number9
StatePublished - Aug 30 2022


  • Fontan
  • Glenn
  • cardiac resynchronization therapy
  • cardiac transplant
  • congenital heart disease
  • electrical dyssynchrony
  • heart failure
  • pediatric
  • single ventricle

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine


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