Some patients with functionally univentricular circulation develop cardiac failure
refractory to maximal management and are supported with a ventricular assist device
(VAD). The purpose of this manuscript is to summarize our previous publications related
to single ventricle-ventricular assist device (sVAD) support in patients with functionally
univentricular circulation and to describe our current institutional approach at University
of Florida to sVAD support in neonates, infants, and children prior to Fontan. Our
programmatic philosophy at University of Florida is to strive to identify the minority
of neonates with functionally univentricular circulation who are extremely high-risk
prior to initiating staged palliation and to stabilize these neonates with primary
preemptive sVAD in preparation for cardiac transplantation; our rationale for this
approach is related to the challenges associated with failed staged palliation and
subsequent bail-out sVAD support and transplantation. A subset of extremely high-risk
neonates and infants with functionally univentricular ductal-dependent circulation
undergo primary preemptive sVAD insertion and subsequent cardiac transplantation.
Support with VAD clearly facilitates survival on the waiting list during prolonged
wait times and optimizes outcomes after Norwood (Stage 1) by providing an alternative
pathway for extremely high-risk patients. Therefore, the selective utilization of
sVAD in extremely high-risk neonates facilitates improved outcomes for all patients
with functionally univentricular ductal-dependent circulation.
At University of Florida, our programmatic approach to utilizing sVAD support as a
bridge to transplantation in the minority of neonates with functionally univentricular
circulation who are extremely high-risk for staged palliation is associated with Operative
Mortality after Norwood (Stage 1) Operation of 2.9% (2/68) and a one-year survival
of 91.1% (82/90) for all neonates presenting with hypoplastic left heart syndrome
(HLHS) or HLHS-related malformation with functionally univentricular ductal-dependent
systemic circulation.
Meanwhile, at University of Florida, for all 82 consecutive neonates, infants, and
children supported with pulsatile paracorporeal VAD: Kaplan-Meier survival estimated
one year after VAD insertion = 73.3% (95% confidence interval [CI] = 64.1–83.8%),
and Kaplan-Meier survival estimated five years after VAD insertion = 68.3% (95% CI
= 58.4–79.8%). For all 48 consecutive neonates, infants, and children at University
of Florida with biventricular circulation supported with pulsatile paracorporeal VAD:
Kaplan-Meier survival estimated one year after VAD insertion = 82.7% (95% CI = 72.4–94.4%),
and Kaplan-Meier survival estimated five years after VAD insertion = 79.7% (95% CI
= 68.6–92.6%). For all 34 consecutive neonates, infants, and children at University
of Florida with functionally univentricular circulation supported with pulsatile paracorporeal
sVAD: Kaplan-Meier survival estimated one year after VAD insertion = 59.7% (95% CI
= 44.9–79.5%), and Kaplan-Meier survival estimated five years after VAD insertion
= 50.5% (95% CI = 35.0–73.0%). These Kaplan-Meier survival estimates for patients
supported with pulsatile paracorporeal VAD are better in patients with biventricular
circulation in comparison to patients with functionally univentricular circulation
both one year after VAD insertion (P=0.026) and five years after VAD insertion (P=0.010).
Although outcomes after VAD support in functionally univentricular patients are worse
than in patients with biventricular circulation, sVAD provides a reasonable chance
for survival. Ongoing research is necessary to improve the outcomes of these challenging
patients, with the goal of developing strategies where outcomes after sVAD support
in functionally univentricular patients are equivalent to the outcomes achieved after
VAD support in patients with biventricular circulation.
Keywords
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Article info
Publication history
Published online: February 02, 2023
Footnotes
Disclosure: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.
Presented at The American Association for Thoracic Surgery (AATS) 102nd Annual Meeting. Saturday, May 14, 2022 to Tuesday, May 17, 2022. John B. Hynes Convention Center, Boston, Massachusetts, United States of America. Presented as an Invited Presentation during: Contemporary Transplant and Mechanical Support. Sunday, May 15, 2022: 8:30 AM - 8:45 AM, Hynes Convention Center, Posted Room Name: Room 302.
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