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)

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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.

Section snippets

INTERMACS database and study population

The Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) is a national prospective database of >16,000 patients in the United States supported on durable MCS devices approved by the Food and Drug Administration. The database is a collaboration between the US National Heart, Lung, and Blood Institute, US Food and Drug Administration, US Centers for Medicare and Medicaid Services, industry, and implantation centers. All registry data are monitored by an independent

Study population

Among 16,182 patients in the INTERMACS database, 126 were identified as having ACHD (Figure 1). Of 158 centers contributing to the INTERMACS registry, only 59 were responsible for all 126 patients, with >70% of centers contributing only 1 or 2 patients (Figure 2). The 6 centers that had the highest volume of ACHD patients on MCS (5 or 6 patients) were high-volume implantation centers with a median non-ACHD implantation volume of 320 patients (range, 236–412 patients) compared with the 41

Discussion

In this article, we present an INTERMACS analysis of the utilization practices, patient characteristics, and outcomes of MCS in patients with ACHD. ACHD patients differed most notably from non-ACHD patients in expected ways, including younger age, greater allosensitization, more right ventricular dysfunction, and unfavorable mediastinal anatomy at time of device implantation. As a group, ACHD patients had higher rates of mortality and adverse events than non-ACHD patients; however, the

Disclosure statement

None of the authors has a financial relationship with a commercial entity that has an interest in the subject of the presented manuscript or other conflicts of interest to disclose.

This study was funded in part by support from the Cardiac Transplant and Education Fund at Boston Children’s Hospital.

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These authors have contributed equally to this work.

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