Paeds Cases · cardiology
Cardiac transplantation and ventricular assist devices — structured clinical encounter
Structured encounter testing the approach to a four-month-old infant with a dilated cardiomyopathy who becomes refractory to medical therapy: recognition of refractory failure, the INTERMACS profile, the durable device decision with the Berlin Heart EXCOR evidence, the transplant-listing prerequisites, and the family counselling.
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You are the paediatric registrar in a tertiary paediatric cardiac centre. A four-month-old infant with a known idiopathic dilated cardiomyopathy and an ejection fraction of 18 percent is on a loop diuretic, an ACE inhibitor and caloric supplementation. Over two weeks the tachypnoea has worsened, feeds take 40 minutes with sweating, the weight has crossed two centiles downward, and the creatinine has begun to rise. The advanced heart-failure team is being consulted. The team asks you to establish that the failure is refractory, to discuss the device pathway, and to begin the family counselling. You have 12 minutes with the team and 5 minutes for examiner discussion. [6]
References4ShowHide
- [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
- [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
- [6]Lasa JJ; Guffey D; Rogers RS; et al Epidemiology and Outcomes of Acute Decompensated Heart Failure in Children. Circ Heart Fail, 2020.PMID 32301336
- [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