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. 2025 May 21.
doi: 10.1097/MAT.0000000000002464. Online ahead of print.

Ventricular Assist Device Use in Pediatric Restrictive and Hypertrophic Cardiomyopathy: An ACTION Registry Analysis

Deepa Mokshagundam  1 , Muhammad F Shezad  2 , Jennifer Conway  3 , John Dykes  4 , Paul Esteso  5 , Ryan Kobayashi  5 , Katsuhide Maeda  6 , Matthew J O'Connor  6 , Jack F Price  7 , Alexander Raskin  8 , Svetlana Shugh  9 , Kathleen E Simpson  10
Affiliations

Ventricular Assist Device Use in Pediatric Restrictive and Hypertrophic Cardiomyopathy: An ACTION Registry Analysis

Deepa Mokshagundam et al. ASAIO J. .

Abstract

The use of ventricular assist devices (VADs) in children with restrictive (RCM) and hypertrophic cardiomyopathy (HCM) remains rare. We describe the outcomes of patients with RCM and HCM supported by VAD in the Advanced Cardiac Therapies Improving Outcomes Network (ACTION) registry from March 2012 to December 2024. Thirty-four patients were identified: 20 left-sided VAD (LVAD), 13 biventricular VAD (BiVAD), and 1 total artificial heart (TAH). Median age at implant was 2.5 years (0.3-17.5), weight was 11.8 kg (4.5-81.8), and body surface area (BSA) was 0.54 m2 (0.26-2.01). Diagnoses included RCM in 25 (73.5%) patients and HCM in 9 (26.5%). Illness severity at implant was high with 38.2% Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) profile 1, 41.2% on extracorporeal membrane oxygenation (ECMO), 52.9% on ventilator support, 88.2% treated with greater than or equal to 1 inotrope, and 52.9% supported with TPN. Four patients (11.8%) died on device, 25 (73.5%) were transplanted, 4 (11.8%) were alive on device, and 1 (2.9%) was transferred to another center. Patients who died all had HCM diagnosis, required BiVAD support, and experienced adverse events. The cause of death was inadequate support (1), infection (2), and multi-organ failure (1). Ventricular assist device support is a reasonable strategy for select RCM and HCM patients, although outcomes in HCM are less favorable.

Keywords: hypertrophic cardiomyopathy; pediatrics; restrictive cardiomyopathy; ventricular assist device.

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Conflict of interest statement

Disclosure: The authors have no conflicts of interest to report.

References

    1. Webber SA, Lipshultz SE, Sleeper LA, et al.; Pediatric Cardiomyopathy Registry Investigators: Outcomes of restrictive cardiomyopathy in childhood and the influence of phenotype. Circulation 126: 1237–1244, 2012.
    1. Amdani S, Boyle G, Saarel E, et al.: Waitlist and post–heart transplant outcomes for children with nondilated cardiomyopathy. Ann Thorac Surg 112: 188–196, 2021.
    1. Magnetta DA, Godown J, West S, et al.: Impact of the 2016 revision of US Pediatric Heart Allocation Policy on waitlist characteristics and outcomes. Am J transplant 19: 3276–3283, 2019.
    1. Lorts A, Smyth L, Gajarski RJ, et al.: The creation of a pediatric health care learning network: The ACTION Quality Improvement Collaborative. ASAIO J 66: 441–446, 2020.
    1. Adachi I, Peng DM, Hollander SA, et al.: Sixth annual Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) report: The Society Of Thoracic Surgeons Pedimacs Annual Report. Ann Thorac Surg 115: 1098–1108, 2023.

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