Paeds · cardiology
Cardiac transplantation and ventricular assist devices
Also known as Cardiac transplantation and ventricular assist devices · Paediatric heart transplantation · Paediatric ventricular assist device · Berlin Heart EXCOR · Mechanical circulatory support in children · Bridge to transplant
Fellowship guide to cardiac transplantation and ventricular assist devices in children: when to escalate from medical heart-failure therapy to mechanical circulatory support, the Berlin Heart EXCOR and continuous-flow durable devices, the INTERMACS urgency profiles, bridge-to-transplant versus bridge-to-recovery strategy, donor allocation and transplant surgery, the denervated transplanted heart, lifelong immunosuppression and surveillance for rejection, cardiac allograft vasculopathy and post-transplant malignancy, and the family pathway through retrieval, listing and long-term follow-up.
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- A child on maximal medical heart-failure therapy who is still symptomatic, losing weight or developing end-organ dysfunction is refractory and must be referred urgently to an advanced heart-failure and transplant centre — the goal of every device and listing decision is to transplant before irreversible pulmonary vascular or renal injury
- An INTERMACS profile 1 (critical cardiogenic shock, 'crash-and-burn') child has the highest short-term mortality on a ventricular assist device — early short-term mechanical circulatory support such as VA-ECMO is used to stabilise the candidate before a durable device or transplant
- Stroke is the dominant complication of the Berlin Heart EXCOR and other paediatric ventricular assist devices and is the leading cause of death on support — a child on a device with a new neurological sign needs urgent imaging and a neurology and haematology review
- A rising pulmonary vascular resistance above the transplant threshold makes a child unsuitable for transplantation, which is why serial catheterisation and timely ventricular assist device unloading matter — irreversible pulmonary hypertension is a contraindication to orthotopic transplantation
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- Cardiac transplantation and ventricular assist devices in children
- Advanced heart failure: indications for mechanical circulatory support and transplantation, device types and complications
- Cardiac transplantation and ventricular assist devices: escalation, INTERMACS profiles, and complications
- Long case: the child with a ventricular assist device awaiting transplantation
- Short case: the transplant survivor on immunosuppression
- Cardiac transplantation and mechanical circulatory support
- Paediatric heart transplantation and ventricular assist devices: indications, devices and complications
- Cardiac: the child on mechanical circulatory support
- Cardiology: heart transplantation and ventricular assist devices
- Patient Care: advanced heart failure, transplantation and mechanical circulatory support
- Medical Expert: heart transplantation and ventricular assist devices in children
Overview & Definition
Cardiac transplantation and ventricular assist devices are the two destinations for the child whose heart failure has become refractory to medical therapy. Transplantation replaces the failing pump with a donor heart; a ventricular assist device (VAD) is a mechanical pump that takes over the work of the ventricle, used to keep the child alive and to restore end-organ function until either the native heart recovers or a donor organ becomes available. Together they are the field of advanced paediatric mechanical circulatory support, and the skill is knowing when to leave medical therapy behind and when to commit to a device and a transplant pathway. [4]
The decision is built on a single principle: a child should be transplanted before irreversible injury to the pulmonary vascular bed, the kidneys or the liver. A ventricular assist device is the instrument that buys the time and reverses the end-organ damage so that the child arrives at transplantation in the best possible condition, and for a subset with myocarditis or a recoverable cardiomyopathy it is itself the cure. The Berlin Heart EXCOR is the established paracorporeal pulsatile pump used as a bridge to transplantation in children including small infants, and miniaturised continuous-flow devices now extend durable support to larger children and adolescents. [1] [11]
References12ShowHide
- [1]Almond CS; Morales DL; Blackstone EH; Turrentine MW; Imamura M; Massicotte MP; Jordan LC; Meyer DM; Das NA; Lorts A; Morales RS; Davies RR; Nikaidoh H; Muenzer JR; Rosenthal DN; Morell VO; Kanter KR; Mahle WT; Fraser CD Jr Berlin Heart EXCOR pediatric ventricular assist device for bridge to heart transplantation in US children. Circulation, 2013.PMID 23538380
- [2]Rosenthal DN; Almond CS; Jaquiss RD; Peyton CE; Auerbach SR; Gomes KM; Blume ED; Pediatric Interagency Registry for Mechanical Circulatory Support (PediMACS) Adverse events in children implanted with ventricular assist devices in the United States: Data from the Pediatric Interagency Registry for Mechanical Circulatory Support (PediMACS). J Heart Lung Transplant, 2016.PMID 27197775
- [3]Blume ED; VanderPluym C; Lorts A; Baldwin JT; Rossano JW; Morales DLS; Cantor RS; Miller MA; Stoddard G; Jaquiss RDB; Rosenthal DN; Kilic A; Auerbach S; Bano M; Bates A; Checcchia P; Fynn-Thompson F; Kirklin JK; PediMACS Investigators Second annual Pediatric Interagency Registry for Mechanical Circulatory Support (PediMACS) report: Pre-implant characteristics and outcomes. J Heart Lung Transplant, 2018.PMID 28965736
- [4]D'Addese L; Boyle G; Spencer R; Gauvreau K; Fynn-Thompson F; Blume E Pediatric heart transplantation in the current era. Curr Opin Pediatr, 2019.PMID 31335745
- [5]Morales DLS; Rossano JW; VanderPluym C; Lorts A; Cantor R; Stoddard G; Adachi I; Almond CS; Bano M; Barta RJ; Bhalla A; Birks EJ; Boyle GJ; Cheng JK; Dreyer WJ; Essa N; Furnary AP; Gurung KJ; Hibino N; Huang JY; John MM; Kanter KR; Kister SE; Kon ZN; Liou A; Maeda K; McDiarmid TN; Mettler BA; Nussbaum DP; Penaranda C; Pigula F; Polito JW; Potapov EV; Schmitz JW; Schowengerdt KO; Smith RP; Thacker LR; Tsui SSL; Uluer AZ; Watkins B; Watson N; Wearden PD; Zwischenberger JB; Koehl DA Fourth Annual Pediatric Interagency Registry for Mechanical Circulatory Support (Pedimacs) Report. Ann Thorac Surg, 2020.PMID 33039359
- [6]Lasa JJ; Guffey D; Rogers RS; Minard CG; Shamszad P; Cabrera AG; Knott-Craig CJ; Goyia CM; Heinle JS; Jefferies JL; Price JF; Mott AR Epidemiology and Outcomes of Acute Decompensated Heart Failure in Children. Circ Heart Fail, 2020.PMID 32301336
- [7]Zimpfer D; Sabol F; Bonatti J; Colah S; De Robertis F; Dimopoulos K; Fiane AE; Gustafsson F; Hartyanszky I; Kaulfersch C; Kiaii B; Kolsrud LIL; Lamm P; Lund LH; Mohacsi P; Musial R; Oliva V; Pereda D; Potapov E; Rega F; Sá MPBO; Schmitto JD; Strüber M; Teutenberg JJ; Veres G; Waraich M; Zeriouh M; Zuckermann A; Sander M Pediatric donor management to optimize donor heart utilization. Pediatr Transplant, 2020.PMID 32198844
- [8]Singh TP; Cherikh WS; Chambers DC; Harhay MN; Hayes D Jr; Potena L; Sadavarte A; Zuckermann A; Khush KK; International Society for Heart and Lung Transplantation The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: Twenty-fourth pediatric heart transplantation report - 2021; focus on recipient characteristics. J Heart Lung Transplant, 2021.PMID 34420853
- [9]Zafar F; Castleberry C; Khan MS; Mehta V; Bryant R III; Dreyer WJ; Adachi I; Fraser CD III; Morales DLS; Lorts A Berlin Heart EXCOR and ACTION post-approval surveillance study report. J Heart Lung Transplant, 2021.PMID 33579597
- [10]Niebler RA; Ghanayem N; VanderPluym C; Lorts A; Adachi I; Bryant R III; Cantor R; Koehl D; Menon S; Bano M; Fraser W; Lasa JJ; Marstein J; Mehta V; Rizwan R; Miller MA; Rosenthal DN; Conway J; Morales DLS Stroke in pediatric ventricular assist device patients-a pedimacs registry analysis. J Heart Lung Transplant, 2021.PMID 33824064
- [11]Lichtenstein KM; Schweiger M; VanderPluym C; Conway J; Schmidt D; Thompson L; Bano M; Zimpfer D Pediatric ventricular assist device registries: update and perspectives in the era of miniaturized continuous-flow pumps. Ann Cardiothorac Surg, 2021.PMID 34159114
- [12]Williams RJ; Taddeo P; Al-Aklabi M; Conway J; D'Astous J; Dipchand AI; L'Heureux A; Mertens L; Porepa LF; Rossano JW; Simpson KE; Singh TP; VanderPluym C; West LJ; McCrindle BW Pediatric heart transplant waiting times in the United States since the 2016 allocation policy change. Am J Transplant, 2022.PMID 34897984