Temporary mechanical circulatory support (tMCS) has been utilized as a bridge to heart transplantation with increasing duration of support. We describe the clinical course of a 14-year-old patient, with TNNT2 and KCNQ1 mutations, requiring Impella 5.5 support for 76 days. We discuss the patient's underlying genetic etiology, potential complications of prolonged tMCS, and the importance of multidisciplinary support. To our knowledge, this is the first published case in which a patient has this specific combination of genetic mutations and the longest published support duration of an Impella 5.5 in a pediatric patient.
Get full access to this article
View all access options for this article.
References
1.
HershbergerREJordanE. Dilated Cardiomyopathy Overview. In: AdamMPFeldmanJMirzaaGMPagonRAWallaceSEAmemiyaA eds. GeneReviews® [Internet].
University of Washington, Seattle;
July 27, 2007:
1993-
2025.
2.
CavaFCristianoEMusumeciMB, et al.TNNI3 and KCNQ1 co-inherited variants in a family with hypertrophic cardiomyopathy and long QT phenotypes: A case report. Mol Genet Metab Rep.
2021;
27, 100743. Published 2021 Mar 18. doi:https://doi.org/10.1016/j.ymgmr.2021.100743.
3.
DimasVVMorrayBHKimDW, et al.A multicenter study of the impella device for mechanical support of the systemic circulation in pediatric and adolescent patients. Catheter Cardiovasc Interv.
2017;
90(
1):
124-
129. doi:https://doi.org/10.1002/ccd.26973
4.
TumeSCFuentes-BaldemarAAAndersM, et al.Temporary ventricular assist device support with a catheter-based axial pump: Changing the paradigm at a pediatric heart center. J Thorac Cardiovasc Surg.
2023;
166(
6):
1756-
1763.e2. doi:https://doi.org/10.1016/j.jtcvs.2022.11.039
5.
BertoldiLFMontisciADelmasCPappalardoF.
Weaning from Impella and mobilization of Impella patients. Eur Heart J Suppl.
2021;
23(
Suppl A):
A41-
A45. Published 2021 Mar 27. doi:https://doi.org/10.1093/eurheartj/suab008
6.
CousinoMKSchumacherKRReaKE, et al.Psychosocial functioning in pediatric heart transplant recipients and their families. Pediatr Transplant.
2018;
22(
2), 10.1111/petr.13110. doi:https://doi.org/10.1111/petr.13110
7.
DiazIThurmCHallM, et al.Disorders of adjustment, mood, and anxiety in children and adolescents undergoing heart transplantation and the association of ventricular assist device support. J Pediatr.
2020;
217:
20-
24.e1. doi:https://doi.org/10.1016/j.jpeds.2019.10.022
