Cardiology Commons^™

A Metabolic, Mechanical, Multi-Organ Masterpiece: Dural Device Support Bridge To En-Bloc Heart-Liver Transplantation In Propionic Acidemia, Rebecca Juhl, Brian Birnbaum, Aliessa P. Barnes, William Gibson, Bhargava Mullapudi, Beth Lang, Megan Faseler, Daniel E. Heble, Victoria Urban, Ryan T. Fischer, Jennifer L. Gannon, David Sutcliffe

Posters

Introduction: Propionic Acidemia (PA) is a disorder related to abnormal protein and lipid metabolism resulting in progressive neurological injury and dilated cardiomyopathy (DCM). Interventions for PA and secondary disease manifestations can require multi-organ transplantation. Herein we report the case of a child with PA and end-stage DCM requiring left ventricular assist device (LVAD) support with eventual heart-liver transplant. Case Report: A 17 year old male diagnosed in childhood with PA developed chronically progressive DCM culminating in end stage heart failure with acute decompensations. In a recurrent admission, he progressed to require dual inotropic support and systemic anticoagulation for new LV …

Knowledge Gap In Adolescent Heart Transplant Recipients, Pamela Finn, Brian Birnbaum, Audrey R. Kennedy, Jennifer A. Marshall, Mark Gelatt

Posters

Background:

 Studies have reported a knowledge gap Studies have reported a knowledge gap among adolescent and young adult heart heart transplant patients upon transitioning to adult cardiology practices.

 This population is vulnerable to life-threatening complications as they progress to adult care.

Framework/Methods:

We assess the current knowledge deficit of our adolescent and young adult patients and their caregivers in order to inform pediatric providers of their unique needs as they prepare to transition.

Heart transplant patients >10 yrs and their caregivers completed a written questionnaire prior to a clinic visit. 17 questions were asked including the need for …