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Paeds Vivascardiology

Paeds Vivas · cardiology

Cardiac transplantation and ventricular assist devices — branching viva

Branching viva from the definition of transplantation and ventricular assist devices through the three-axis classification, the INTERMACS urgency profiles, the Berlin Heart EXCOR evidence, the denervated transplanted heart, and the complications that dominate each stage.

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Target exams

RACP General PaediatricsRACP DCEMRCPCH ClinicalRCPSC Pediatrics

Target exams

RACP General PaediatricsRACP DCEMRCPCH ClinicalRCPSC Pediatrics
Prompt
You are the paediatric registrar in a tertiary cardiac centre. The consultant asks you to talk through four children: a four-month-old with a refractory dilated cardiomyopathy being assessed for a device, a two-year-old who has collapsed in critical cardiogenic shock, a ten-year-old on a Berlin Heart EXCOR who develops a new neurological sign, and a sixteen-year-old transplant recipient attending for long-term follow-up.

Station opening

Examiner: "Define cardiac transplantation and ventricular assist devices, and explain when they enter a child's care." [4]

Strong candidate (must-hit)

  • Defines transplantation as the definitive replacement of the failing heart with a donor organ, and a ventricular assist device as a mechanical pump that unloads and supports the failing ventricle; explains that they are the two destinations for the child whose heart failure has become refractory to medical therapy — a VAD used as a bridge to transplantation (most), a bridge to recovery (myocarditis and selected cardiomyopathy), a bridge to candidacy, or rarely destination therapy; and frames the whole pathway as a race to transplant before irreversible end-organ or pulmonary vascular injury. [4]

Weak candidate

  • "A VAD is a pump we put in when the heart fails, and transplant is when we give a new heart." [4]

Branch A — The infant with a refractory dilated cardiomyopathy

Examiner: "A four-month-old with a dilated cardiomyopathy is worsening despite a diuretic, an ACE inhibitor and caloric supplementation, and the creatinine is rising. How do you classify the support options, and what device is most likely?" [1]

Strong

  • Classifies mechanical support on three axes — device type, INTERMACS urgency profile, and strategic goal; identifies this child as refractory and likely INTERMACS profile 2 or 3 (declining or stable on inotropes); selects the durable paracorporeal pulsatile Berlin Heart EXCOR because its pump sizes reach infants, citing the Almond 2013 cohort that established its bridge-to-transplant role; and states that the strategic goal is bridge to transplantation, with serial catheterisation of the pulmonary vascular resistance before listing. [1] [4]

Weak

  • "Put her on ECMO and wait for a heart." [1]

Branch B — The two-year-old in critical cardiogenic shock

Examiner: "A two-year-old who was previously well is brought in grey, mottled and poorly perfused with a lactate of 9 and weak pulses, deteriorating despite two inotropes. What is happening, what INTERMACS profile is this, and what is your first escalation?" [3]

Strong

  • Classifies this as critical cardiogenic shock — the INTERMACS profile 1 'crash-and-burn' patient with the highest early mortality on support; explains that the first escalation is short-term mechanical circulatory support with VA-ECMO to restore perfusion and prevent irreversible end-organ death, used as a stabilising bridge to a durable device or transplant; and states that ECMO is a bridge to a decision (recovery, durable VAD, or transplantation), not a destination, so an early exit strategy must be defined. [3]

Weak

  • "Give a fluid bolus and more inotropes and see if she improves." [3]

Branch C — The child on a Berlin Heart EXCOR with a new neurological sign

Examiner: "A ten-year-old has been on a Berlin Heart EXCOR awaiting transplant for three weeks. The nurse finds a new left-sided weakness. What is the diagnosis, why is it feared, and what do you do?" [10]

Strong

  • Diagnoses a stroke until proven otherwise — the dominant and most feared complication of paediatric VADs and a leading cause of death on support, quantified by the PediMACS stroke analysis; secures ABC, obtains urgent brain imaging (CT then MRI) to distinguish ischaemic from haemorrhagic stroke, notifies the advanced heart-failure, neurology and haematology teams, and adjusts anticoagulation according to the stroke type and bleeding risk; and explains that anticoagulation on a VAD is mandatory but unforgiving, balancing pump thrombosis against bleeding. [10]

Weak

  • "It's probably sedation; check the observations and review in the morning." [10]

Branch D — The transplant recipient in long-term follow-up

Examiner: "A sixteen-year-old is three years after transplantation for a cardiomyopathy. Describe the denervated heart, the immunosuppression, and the complications you are watching for." [8]

Strong

  • Explains that the donor heart is denervated — a resting tachycardia, a blunted chronotropic response to exercise, and atropine is ineffective; describes lifelong triple immunosuppression with a calcineurin inhibitor, an antiproliferative agent and a corticosteroid taper; and identifies the complications under surveillance: acute cellular and antibody-mediated rejection (diagnosed on surveillance endomyocardial biopsy), cardiac allograft vasculopathy (the leading cause of late graft loss, screened by imaging), post-transplant lymphoproliferative disease (EBV-driven), and calcineurin-inhibitor renal toxicity; adds that adolescent adherence and structured transition to adult care are central. [4] [8]

Weak

  • "She takes tacrolimus and we check her bloods once a year." [8]

Close

Examiner: "Summarise your approach to advanced paediatric heart failure in one sentence." [4]

Strong

  • "Cardiac transplantation and ventricular assist devices are the two destinations for the child with refractory heart failure: I escalate early — before irreversible end-organ injury — because the whole pathway is a race to transplant the child in the best possible condition; I classify support by device type, the INTERMACS urgency profile, and the strategic goal; I stabilise the profile-1 child with VA-ECMO, choose a durable device by the size of the child, and transplant with lifelong triple immunosuppression — watching for stroke and infection on the device, and rejection, cardiac allograft vasculopathy, post-transplant malignancy and renal toxicity after transplant." [4]

References

  1. [1]Almond CS; Morales DL; Blackstone EH; et al Berlin Heart EXCOR pediatric ventricular assist device for bridge to heart transplantation in US children. Circulation, 2013.PMID 23538380
  2. [3]Blume ED; VanderPluym C; Lorts A; et al Second annual Pediatric Interagency Registry for Mechanical Circulatory Support (PediMACS) report: Pre-implant characteristics and outcomes. J Heart Lung Transplant, 2018.PMID 28965736
  3. [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
  4. [8]Singh TP; Cherikh WS; Chambers DC; et al 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
  5. [10]Niebler RA; Ghanayem N; VanderPluym C; et al Stroke in pediatric ventricular assist device patients-a pedimacs registry analysis. J Heart Lung Transplant, 2021.PMID 33824064