Author + information
- Received March 23, 2020
- Revision received May 27, 2020
- Accepted May 28, 2020
- Published online July 20, 2020.
- Jiyong Moon, MDa,b,
- Li Shen, MDa,c,
- Donald S. Likosky, PhDd,
- Vikram Sood, MDa,
- Reilly D. Hobbs, MD, MBSa,
- Peter Sassalos, MDa,
- Jennifer C. Romano, MDa,
- Richard G. Ohye, MDa,
- Edward L. Bove, MDa and
- Ming-Sing Si, MDa,∗ (, )@UMich
- aSection of Pediatric Cardiovascular Surgery, Department of Cardiac Surgery, University of Michigan Medical School, University of Michigan, C.S. Mott Children’s Hospital, Ann Arbor, Michigan
- bDivision of Congenital Heart Surgery, Department of Surgery, Baylor College of Medicine Texas Children’s Hospital, Houston, Texas
- cDepartment of Cardiothoracic Surgery, Shanghai Jiaotong University, Shanghai Children Hospital, Shanghai, China
- dDepartment of Cardiac Surgery, Section of Health Services Research and Quality, University of Michigan Medical School, University of Michigan, Ann Arbor, Michigan
- ↵∗Address for correspondence:
Dr. Ming-Sing Si, University of Michigan Congenital Heart Center, C. S. Mott Children's Hospital, 1540 E. Hospital Drive, SPC 4284, Ann Arbor, Michigan 48109–4284.
Background The influence of ventricular morphology on Fontan outcomes is controversial.
Objectives This study hypothesized that dysfunction of the single right ventricle (RV) and right atrioventricular valve regurgitation (AVVR) increases over time and adversely impacts late outcomes following a Fontan operation. A single-center retrospective study was performed.
Methods From 1985 through 2018, 1,162 patients underwent the Fontan procedure at our center and were included in this study. Transplant and takedown free survival, ventricular, and atrioventricular valve dysfunction after Fontan were analyzed. Death or heart transplantation information was obtained from the National Death Index and the Scientific Registry of Transplant Recipients.
Results The follow-up rate was 99%. Morphologic RV was present in 58% of patients. Transplant and takedown free survival were 91%, 75%, and 71% at 10 years, 20 years, and 25 years, respectively. Morphologic RV was an independent risk factor for transplant, takedown free survival (hazard ratio: 2.4; p = 0.008). The AVVR, which preceded ventricular dysfunction in most cases, was associated with the development of ventricular dysfunction after Fontan (odds ratio: 4.3; 95% confidence interval: 2.7 to 6.7; p < 0.001). Furthermore, AVVR and ventricular dysfunction progressed over time after Fontan, especially in the RV (AVVR: p < 0.0001, ventricular dysfunction: p < 0.0001).
Conclusions Morphologic RV is negatively associated with the long-term survival following the Fontan, possibly due to a tendency toward progressive AVVR and deterioration of the single ventricle function. Additional volume overload caused by AVVR may be one of the main factors accelerating the dysfunction of the single RV, implying that early valve intervention may be warranted.
Dr. Si has received funding from the National Heart, Lung, and Blood Institute (NHLBI) (K08HL146351). Dr. Likosky has received funding from the Agency for Healthcare Research and Quality (AHRQ; R01HS026003) and NHLBI (HL146619–01A1). Opinions expressed in this manuscript do not represent those of NHLBI, AHRQ, or the U.S. Department of Health and Human Services. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose. The data reported here have been supplied by the Hennepin Healthcare Research Institute as the contractor for the Scientific Registry of Transplant Recipients (SRTR). The interpretation and reporting of these data are the responsibility of the author(s) and in no way should be seen as an official policy of or interpretation by the SRTR or the U.S. government.
The authors attest they are in compliance with human studies committees and animal welfare regulations of the authors’ institutions and Food and Drug Administration guidelines, including patient consent where appropriate. For more information, visit the JACC author instructions page.
- Received March 23, 2020.
- Revision received May 27, 2020.
- Accepted May 28, 2020.
- 2020 American College of Cardiology Foundation
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