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 Czosek; Ming-Lon Young; David J Bradley; Thomas Paul; Peter Fischbach; Lindsey Malloy-Walton; Doff B McElhinney; Anne M Dubin

doi:10.1016/j.jacc.2022.05.053

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

J Am Coll Cardiol. 2022 Aug 30;80(9):902-914. doi: 10.1016/j.jacc.2022.05.053.

Authors

Henry Chubb¹, Anica Bulic², Douglas Mah³, Jeremy P Moore⁴, Jan Janousek⁵, Jennifer Fumanelli⁶, S Yukiko Asaki⁷, Andreas Pflaumer⁸, Allison C Hill⁹, Carolina Escudero¹⁰, Sit Yee Kwok¹¹, Jasveer Mangat¹², Luis A Ochoa Nunez¹³, Seshadri Balaji¹⁴, Eric Rosenthal¹⁵, William Regan¹⁵, Michaela Horndasch¹⁶, Hiroko Asakai¹⁷, Ronn Tanel¹⁸, Richard J Czosek¹⁹, Ming-Lon Young²⁰, David J Bradley²¹, Thomas Paul²², Peter Fischbach²³, Lindsey Malloy-Walton²⁴, Doff B McElhinney²⁵, Anne M Dubin²⁶

Affiliations

¹ Division of Pediatric Cardiology, Department of Pediatrics, Stanford University, Stanford, California, USA; Division of Pediatric Cardiothoracic Surgery, Department of Cardiothoracic Surgery, Stanford University, Stanford, California, USA. Electronic address: mhchubb@stanford.edu.
² Labatt Family Heart Centre, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
³ Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts, USA; Department of Pediatrics, Harvard Medical School, Boston, Massachusetts, USA.
⁴ Division of Cardiology, Department of Pediatrics, UCLA Health System, Los Angeles, California, USA; Division of Cardiology, Department of Medicine, Ahmanson/UCLA Adult Congenital Heart Disease Center, Los Angeles, California, USA; UCLA Cardiac Arrhythmia Center, UCLA Health System, Los Angeles, California, USA.
⁵ Children's Heart Centre, Second Faculty of Medicine, Charles University in Prague and Motol University Hospital, Prague, Czech Republic.
⁶ Children's Heart Centre, Second Faculty of Medicine, Charles University in Prague and Motol University Hospital, Prague, Czech Republic; Pediatric Cardiology Unit, Department of Women's and Child's Health, University of Padova, Padova, Italy.
⁷ Primary Children's Hospital, University of Utah, Salt Lake City, Utah, USA.
⁸ The Royal Children's Hospital, MCRI and University of Melbourne, Melbourne, Victoria, Australia.
⁹ Division of Cardiology, Children's Hospital Los Angeles, Los Angeles, California, USA; Keck School of Medicine, University of Southern California, Los Angeles, California, USA.
¹⁰ Department of Pediatrics, Division of Pediatric Cardiology, University of Alberta, Stollery Children's Hospital, Edmonton, Alberta, Canada.
¹¹ Cardiology Centre, Department of Paediatrics and Adolescent Medicine, Hong Kong Children's Hospital, Hong Kong SAR, China.
¹² Paediatric Cardiology, Great Ormond Street, London, United Kingdom.
¹³ University of Iowa Healthcare, Iowa City, Iowa, USA.
¹⁴ Department of Pediatrics, Division of Cardiology, Oregon Health & Science University, Portland, Oregon, USA.
¹⁵ Paediatric Cardiology, Evelina London Children's Hospital, London, United Kingdom.
¹⁶ Department of Congenital Heart Diseases and Pediatric Cardiology, German Heart Center Munich, Munich, Germany.
¹⁷ Department of Paediatrics, University of Tokyo Hospital, Tokyo, Japan.
¹⁸ Division of Pediatric Cardiology, Department of Pediatrics, UCSF School of Medicine, San Francisco, California, USA.
¹⁹ The Heart Institute, Cincinnati Children's Hospital Medical Center, Ohio, USA.
²⁰ Joe DiMaggio Children's Hospital, Hollywood, Florida, USA.
²¹ University of Michigan, CS Mott Children's Hospital, Ann Arbor, Michigan, USA.
²² Department of Pediatric Cardiology, Georg-August-University Medical Center, Göttingen, Germany.
²³ Sibley Heart Center, Atlanta, Georgia, USA.
²⁴ Children's Mercy Hospital, University of Missouri, Kansas City, Missouri, USA.
²⁵ Division of Pediatric Cardiology, Department of Pediatrics, Stanford University, Stanford, California, USA; Division of Pediatric Cardiothoracic Surgery, Department of Cardiothoracic Surgery, Stanford University, Stanford, California, USA.
²⁶ Division of Pediatric Cardiology, Department of Pediatrics, Stanford University, Stanford, California, USA.

PMID: 36007989
DOI: 10.1016/j.jacc.2022.05.053

Abstract

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 (PPM_v) may be associated with additional adverse long-term outcomes.

Objectives: The goal of this study was to quantify the attributable risk of PPM_v 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 PPM_v. Enrollment was at implantation. Controls were matched 1:1 to PPM_v subjects by ventricular morphology and sex, identified within center, and enrolled at matched age. Primary outcome was transplantation or death.

Results: In total, 236 PPM_v 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 PPM_v cohort. Across 213 matched pairs, multivariable HR for death/transplant associated with PPM_v was 3.8 (95% CI 1.9-7.6; P < 0.001). Within the PPM_v 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: PPM_v in patients with SV is associated with increased risk of heart transplantation and death, despite controlling for increased associated morbidity of the PPM_v 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.

Keywords: Fontan; Glenn; cardiac resynchronization therapy; cardiac transplant; congenital heart disease; electrical dyssynchrony; heart failure; pediatric; single ventricle.

Publication types

Research Support, Non-U.S. Gov't

MeSH terms

Child
Cohort Studies
Heart Defects, Congenital*
Heart Transplantation*
Heart Ventricles
Humans
Retrospective Studies
Treatment Outcome
Univentricular Heart*