Christina J VanderPluym1, Ari Cedars2, Pirooz Eghtesady3, Bryan G Maxwell4, Jill M Gelow5, Luke J Burchill5, Simon Maltais6, Devin A Koehl7, Ryan S Cantor8, Elizabeth D Blume9. 1. Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts. Electronic address: Christina.vanderpluym@childrens.harvard.edu. 2. Department of Cardiology, Baylor University Medical Center, Dallas, Texas. 3. Department of Cardiothoracic Surgery, Washington University School of Medicine, St. Louis, Missouri. 4. Department of Anesthesiology, Randall Children's Hospital, Portland, Oregon. 5. Knight Cardiovascular Institute, Oregon Health and Science University, Portland, Oregon. 6. Department of Cardiovascular Surgery, Mayo Clinic School of Medicine, Rochester, Minnesota. 7. Department of Cardiothoracic Surgery, University of Alabama at Birmingham, Birmingham, Alabama. 8. Department of Cardiothoracic Surgery, University of Alabama at Birmingham, Birmingham, Alabama; Department of Epidemiology, School of Public Health, University of Alabama at Birmingham, Birmingham, Alabama. 9. Department of Cardiology, Boston Children's Hospital, Boston, Massachusetts.
Abstract
BACKGROUND: Adults with congenital heart disease represent an expanding and unique population of patients with heart failure (HF) in whom the use of mechanical circulatory support (MCS) has not been characterized. We sought to describe overall use, patient characteristics, and outcomes of MCS in adult congenital heart disease (ACHD). METHODS: All patients entered into the Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) between June 23, 2006, and December 31, 2015, were included. Patients with ACHD were identified using pre-operative data and stratified by ventricular morphology. Mortality was compared between ACHD and non-ACHD patients, and multivariate analysis was performed to identify predictors of death after device implantation. RESULTS: Of 16,182 patients, 126 with ACHD stratified as follows: systemic morphologic left ventricle (n = 63), systemic morphologic right ventricle (n = 45), and single ventricle (n = 17). ACHD patients were younger (42 years ± 14 vs 56 years ± 13; p < 0.0001) and were more likely to undergo device implantation as bridge to transplant (45% vs 29%; p < 0.0001). A higher proportion of ACHD patients had biventricular assist device (BiVAD)/total artificial heart (TAH) support compared with non-ACHD patients (21% vs 7%; p < 0.0001). More ACHD patients on BiVAD/TAH support were INTERMACS profile 1 compared with patients on systemic left ventricular assist device (LVAD) support (35% vs 15%; p = 0.002). ACHD and non-ACHD patients with LVADs had similar survival; survival was worse for patients on BIVAD/TAH support. BiVAD/TAH support was the only variable independently associated with mortality (early phase hazard ratio 4.4; 95% confidence interval, 1.8-11.1; p = 0.001). For ACHD patients receiving MCS, ventricular morphology was not associated with mortality. CONCLUSIONS: ACHD patients with LVADs have survival similar to non-ACHD patients. Mortality is higher for patients requiring BiVAD/TAH support, potentially owing to higher INTERMACS profile. These outcomes suggest a promising role for LVAD support in ACHD patients as part of the armamentarium of therapies for advanced HF.
BACKGROUND: Adults with congenital heart disease represent an expanding and unique population of patients with heart failure (HF) in whom the use of mechanical circulatory support (MCS) has not been characterized. We sought to describe overall use, patient characteristics, and outcomes of MCS in adult congenital heart disease (ACHD). METHODS: All patients entered into the Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) between June 23, 2006, and December 31, 2015, were included. Patients with ACHD were identified using pre-operative data and stratified by ventricular morphology. Mortality was compared between ACHD and non-ACHD patients, and multivariate analysis was performed to identify predictors of death after device implantation. RESULTS: Of 16,182 patients, 126 with ACHD stratified as follows: systemic morphologic left ventricle (n = 63), systemic morphologic right ventricle (n = 45), and single ventricle (n = 17). ACHD patients were younger (42 years ± 14 vs 56 years ± 13; p < 0.0001) and were more likely to undergo device implantation as bridge to transplant (45% vs 29%; p < 0.0001). A higher proportion of ACHD patients had biventricular assist device (BiVAD)/total artificial heart (TAH) support compared with non-ACHD patients (21% vs 7%; p < 0.0001). More ACHD patients on BiVAD/TAH support were INTERMACS profile 1 compared with patients on systemic left ventricular assist device (LVAD) support (35% vs 15%; p = 0.002). ACHD and non-ACHD patients with LVADs had similar survival; survival was worse for patients on BIVAD/TAH support. BiVAD/TAH support was the only variable independently associated with mortality (early phase hazard ratio 4.4; 95% confidence interval, 1.8-11.1; p = 0.001). For ACHD patients receiving MCS, ventricular morphology was not associated with mortality. CONCLUSIONS: ACHD patients with LVADs have survival similar to non-ACHD patients. Mortality is higher for patients requiring BiVAD/TAH support, potentially owing to higher INTERMACS profile. These outcomes suggest a promising role for LVAD support in ACHD patients as part of the armamentarium of therapies for advanced HF.
Authors: Rhoia Neidenbach; Koichiro Niwa; Oeztekin Oto; Erwin Oechslin; Jamil Aboulhosn; David Celermajer; Joerg Schelling; Lars Pieper; Linda Sanftenberg; Renate Oberhoffer; Fokko de Haan; Michael Weyand; Stephan Achenbach; Christian Schlensak; Dirk Lossnitzer; Nicole Nagdyman; Yskert von Kodolitsch; Hans-Carlo Kallfelz; David Pittrow; Ulrike M M Bauer; Peter Ewert; Thomas Meinertz; Harald Kaemmerer Journal: Cardiovasc Diagn Ther Date: 2018-12
Authors: Elvin Zengin; Christoph Sinning; Christopher Blaum; Stefan Blankenberg; Carsten Rickers; Yskert von Kodolitsch; Paulus Kirchhof; Nigel E Drury; Victoria M Stoll Journal: Cardiovasc Diagn Ther Date: 2021-04
Authors: David M Peng; Devin A Koehl; Ryan S Cantor; Kristen N McMillan; Aliessa P Barnes; Patrick I McConnell; Jessica Jordan; Nicholas D Andersen; James D St Louis; Katsuhide Maeda; James K Kirklin; Steven J Kindel Journal: J Heart Lung Transplant Date: 2018-10-31 Impact factor: 10.247