Laith I Alshawabkeh1, Nan Hu2, Knute D Carter2, Alexander R Opotowsky3, KellyAnn Light-McGroary4, Joseph E Cavanaugh2, Heather L Bartlett5. 1. Boston Adult Congenital Heart Program (BACH) at the Department of Cardiology, Boston Children's Hospital, and Department of Medicine, Division of Cardiology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts; Division of Cardiovascular Medicine, Department of Internal Medicine, Carver College of Medicine, Iowa City, Iowa. Electronic address: laith.alshawabkeh@tch.harvard.edu. 2. Department of Biostatistics, College of Public Health, University of Iowa, Iowa City, Iowa. 3. Boston Adult Congenital Heart Program (BACH) at the Department of Cardiology, Boston Children's Hospital, and Department of Medicine, Division of Cardiology, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts. 4. Division of Cardiovascular Medicine, Department of Internal Medicine, Carver College of Medicine, Iowa City, Iowa. 5. Department of Pediatrics and Medicine, School of Medicine and Public Health, University of Wisconsin, Madison, Wisconsin.
Abstract
BACKGROUND: Heart failure represents a common end-stage syndrome for many adults with congenital heart disease (ACHD). These patients, however, have been excluded from most heart transplantation research. It is not known how current criteria, derived from non-ACHD populations, used to determine priority at the time of transplant listing, impact the outcomes for ACHD patients listed for heart transplantation. OBJECTIVES: The goal of this study was to investigate outcomes of ACHD in comparison to non-ACHD patients while listed for heart transplantation. METHODS: We conducted a retrospective study using the Scientific Registry of Transplant Recipients on patients ≥18 years of age listed in the United States between 1999 and 2014. The probability of mortality or delisting due to clinical worsening was estimated using cumulative incidence functions, where transplantation was a competing event. RESULTS: Among 1,290 ACHD and 38,557 non-ACHD patients listed, 237 ACHD and 6,377 non-ACHD patients died or were delisted due to clinical worsening. Death or delisting for clinical worsening was more likely for ACHD patients initially listed as status 1A (24% ACHD vs. 17% non-ACHD after 180 days; p < 0.001). There were no significant differences between ACHD and non-ACHD patients listed as status 1B or 2. In multivariable analysis, factors associated with death or delisting due to clinical worsening within 1 year in ACHD included: estimated glomerular filtration rate <60 ml/min/1.73 m(2) (hazard ratio [HR]: 1.4; 95% confidence interval [CI]: 1.0 to 1.9; p = 0.043); albumin <3.2 g/dl (HR: 2.0; 95% CI: 1.3 to 2.9; p <0.001); and hospitalization at the time of listing, whether in the intensive care unit (HR: 2.3; 95% CI: 1.6 to 3.5; p < 0.001) or not (HR: 1.9; 95% CI: 1.2 to 3.0; p = 0.006) relative to outpatients. CONCLUSIONS: Wait-list mortality or delisting due to worsening clinical status is disproportionately common for ACHD patients listed as status 1A. An allocation system that takes into account the distinctive aspects of ACHD patients may help better care for this growing population.
BACKGROUND:Heart failure represents a common end-stage syndrome for many adults with congenital heart disease (ACHD). These patients, however, have been excluded from most heart transplantation research. It is not known how current criteria, derived from non-ACHD populations, used to determine priority at the time of transplant listing, impact the outcomes for ACHD patients listed for heart transplantation. OBJECTIVES: The goal of this study was to investigate outcomes of ACHD in comparison to non-ACHD patients while listed for heart transplantation. METHODS: We conducted a retrospective study using the Scientific Registry of Transplant Recipients on patients ≥18 years of age listed in the United States between 1999 and 2014. The probability of mortality or delisting due to clinical worsening was estimated using cumulative incidence functions, where transplantation was a competing event. RESULTS: Among 1,290 ACHD and 38,557 non-ACHD patients listed, 237 ACHD and 6,377 non-ACHD patients died or were delisted due to clinical worsening. Death or delisting for clinical worsening was more likely for ACHD patients initially listed as status 1A (24% ACHD vs. 17% non-ACHD after 180 days; p < 0.001). There were no significant differences between ACHD and non-ACHD patients listed as status 1B or 2. In multivariable analysis, factors associated with death or delisting due to clinical worsening within 1 year in ACHD included: estimated glomerular filtration rate <60 ml/min/1.73 m(2) (hazard ratio [HR]: 1.4; 95% confidence interval [CI]: 1.0 to 1.9; p = 0.043); albumin <3.2 g/dl (HR: 2.0; 95% CI: 1.3 to 2.9; p <0.001); and hospitalization at the time of listing, whether in the intensive care unit (HR: 2.3; 95% CI: 1.6 to 3.5; p < 0.001) or not (HR: 1.9; 95% CI: 1.2 to 3.0; p = 0.006) relative to outpatients. CONCLUSIONS: Wait-list mortality or delisting due to worsening clinical status is disproportionately common for ACHD patients listed as status 1A. An allocation system that takes into account the distinctive aspects of ACHD patients may help better care for this growing population.
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