Highlights
- •The ventricular assist device (VAD) therapy is getting common in advanced heart failure treatment for bridge to transplant and destination therapy.
- •The adult congenital heart disease (ACHD) patients with advanced heart failure had less access to the VAD therapy and the heart transplant in the past.
- •The ACHD patients have similar survival and improvement of quality of life after left VAD implantation compared to non-ACHD patients.
Abstract
As long-term surgical outcome of congenital heart disease has continued to improve,
most pediatric patients with congenital heart disease are able to reach adulthood.
However, adult congenital heart disease (ACHD) patients have increased risk of arrhythmia,
valvular diseases, infectious endocarditis, and heart failure. The end-stage ACHD
patients with advanced heart failure may require mechanical circulatory support to
improve the heart failure symptoms or to recover from circulatory collapse, and may
eventually aim to heart transplant or destination therapy. In general, long-term mechanical
support for dilated cardiomyopathy or ischemic cardiomyopathy has been achieved with
left ventricular assist device with excellent survival outcomes and improved quality
of life. However, the ventricular assist device for end-stage ACHD patients can be
challenging due to patient-specific anatomical feature, multiple histories of surgical
and catheter-based interventions and possible multiple end-organ dysfunctions, and
offered less frequently compared to non-ACHD patients.
The Interagency Registry for Mechanically Assisted Circulatory Support data published
recently showed that ACHD patients receiving long-term mechanical circulatory support
consisted <1 % of all registrants and had higher mortality after mechanical support
than non-ACHD patients. However, the ACHD patients supported with left ventricular
assist device had similar survival with non-ACHD patients and a large proportion of
the mortality difference between ACHD and non-ACHD patients seemed to result from
operative and perioperative factors. Therefore, the ventricular assist device therapy
can be an excellent treatment for selected ACHD patients.
In this paper, we describe the current status of ventricular assist device support
for end-stage ACHD patients and consideration to the future.
Graphical abstract

Graphical Abstract
Keywords
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Article info
Publication history
Published online: October 28, 2022
Accepted:
September 14,
2022
Received:
September 2,
2022
Identification
Copyright
© 2022 Japanese College of Cardiology. Published by Elsevier Ltd. All rights reserved. All rights reserved.