Document Detail

Left Ventricular Noncompaction Cardiomyopathy in Barth Syndrome: An Example of an Undulating Cardiac Phenotype Necessitating Mechanical Circulatory Support as a Bridge to Transplantation.
MedLine Citation:
PMID:  22427193     Owner:  NLM     Status:  Publisher    
Barth syndrome (BTHS) is associated with myocardial disease, frequently left ventricular noncompaction cardiomyopathy, which may necessitate cardiac transplantation or lead to death in some patients. We report a child with BTHS who had an "undulating cardiac phenotype" and ultimately developed decompensated heart failure requiring mechanical circulatory support with a ventricular assist device as a bridge to transplantation. His course was complicated by acute lung injury requiring placement of an in-line oxygenator to maintain end-organ function. Not only was his course complicated by cardiac and respiratory failure but his BTHS associated comorbidities complicated the management of his therapy using mechanical assist device support. He was successfully supported and subsequently was transplanted. Here we discuss the management of a child with BTHS using mechanical circulatory support and describe the use of an in-line oxygenator, Quadrox, with the Berlin Excor device.
Samuel P Hanke; Aimee B Gardner; John P Lombardi; Peter B Manning; David P Nelson; Jeffrey A Towbin; John L Jefferies; Angela Lorts
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-3-17
Journal Detail:
Title:  Pediatric cardiology     Volume:  -     ISSN:  1432-1971     ISO Abbreviation:  -     Publication Date:  2012 Mar 
Date Detail:
Created Date:  2012-3-19     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8003849     Medline TA:  Pediatr Cardiol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
The Heart Institute, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue-ML 2003, Cincinnati, OH, 45213, USA.
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