Outcomes following implantation of mechanical circulatory support in adults with congenital heart disease: An analysis of the Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS)

Christina J. VanderPluym, Ari Cedars, Pirooz Eghtesady, Bryan G. Maxwell, Jill M. Gelow, Luke J. Burchill, Simon Maltais, Devin A. Koehl, Ryan S. Cantor, Elizabeth D. Blume

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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.

Original languageEnglish (US)
Pages (from-to)89-99
Number of pages11
JournalJournal of Heart and Lung Transplantation
Volume37
Issue number1
DOIs
StatePublished - Jan 1 2018

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Registries
Heart Diseases
Artificial Heart
Equipment and Supplies
Heart-Assist Devices
Mortality
Heart Ventricles
Survival
Heart Failure

Keywords

  • congenital heart disease
  • LVAD
  • mechanical circulatory support
  • single ventricle
  • total artificial heart
  • ventricular assist device

ASJC Scopus subject areas

  • Surgery
  • Pulmonary and Respiratory Medicine
  • Cardiology and Cardiovascular Medicine
  • Transplantation

Cite this

Outcomes following implantation of mechanical circulatory support in adults with congenital heart disease : An analysis of the Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS). / VanderPluym, Christina J.; Cedars, Ari; Eghtesady, Pirooz; Maxwell, Bryan G.; Gelow, Jill M.; Burchill, Luke J.; Maltais, Simon; Koehl, Devin A.; Cantor, Ryan S.; Blume, Elizabeth D.

In: Journal of Heart and Lung Transplantation, Vol. 37, No. 1, 01.01.2018, p. 89-99.

Research output: Contribution to journalArticle

VanderPluym, Christina J. ; Cedars, Ari ; Eghtesady, Pirooz ; Maxwell, Bryan G. ; Gelow, Jill M. ; Burchill, Luke J. ; Maltais, Simon ; Koehl, Devin A. ; Cantor, Ryan S. ; Blume, Elizabeth D. / Outcomes following implantation of mechanical circulatory support in adults with congenital heart disease : An analysis of the Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS). In: Journal of Heart and Lung Transplantation. 2018 ; Vol. 37, No. 1. pp. 89-99.
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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.",
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author = "VanderPluym, {Christina J.} and Ari Cedars and Pirooz Eghtesady and Maxwell, {Bryan G.} and Gelow, {Jill M.} and Burchill, {Luke J.} and Simon Maltais and Koehl, {Devin A.} and Cantor, {Ryan S.} and Blume, {Elizabeth D.}",
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T1 - Outcomes following implantation of mechanical circulatory support in adults with congenital heart disease

T2 - An analysis of the Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS)

AU - VanderPluym, Christina J.

AU - Cedars, Ari

AU - Eghtesady, Pirooz

AU - Maxwell, Bryan G.

AU - Gelow, Jill M.

AU - Burchill, Luke J.

AU - Maltais, Simon

AU - Koehl, Devin A.

AU - Cantor, Ryan S.

AU - Blume, Elizabeth D.

PY - 2018/1/1

Y1 - 2018/1/1

N2 - 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.

AB - 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.

KW - congenital heart disease

KW - LVAD

KW - mechanical circulatory support

KW - single ventricle

KW - total artificial heart

KW - ventricular assist device

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