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Cardiac Transplantation in a Pediatric Patient with Systemic Sclerosis.
MedLine Citation:
PMID:  21801316     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Introduction.  Diffuse cutaneous systemic sclerosis (SSc) is rare in children, but has a poor prognosis when cardiomyopathy is present. Methods.  We reviewed the case of a 14-year-old female with progressive skin thickening/tightness and dyspnea on exertion who was diagnosed with SSc. Results.  Our patient was found to have severe restrictive cardiomyopathy with poor left ventricular systolic function (ejection fraction = 20%), unresponsive to the immunosuppression used to treat her SSc. There was no evidence of pulmonary fibrosis. The patient underwent orthotopic cardiac transplantation, with improvement in systemic symptoms. Two years after transplantation, she had elevated filling pressures during a surveillance catheterization, with no evidence of cellular rejection or coronary artery vasculopathy. Four months later, she developed severe ventricular dysfunction and dyspnea, despite a negative biopsy and negative C4d immunofluorescence staining. Her immunosuppression was intensified with improvement of cardiac function. Despite this, 1 month later she had sudden pulseless cardiac arrest from which she could not be revived. The family declined an autopsy. Discussion.  We report a 14-year-old female with SSc who had cardiac transplantation. The etiology behind her recurrent restrictive physiology, cardiac dysfunction, and subsequent cardiac arrest remains unclear.
Authors:
Jonathan N Johnson; Bonnie S Kvistad; Ann M Reed; Co-Burn J Porter; Patrick W O'Leary; David J Driscoll
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2011-7-31
Journal Detail:
Title:  Congenital heart disease     Volume:  -     ISSN:  1747-0803     ISO Abbreviation:  -     Publication Date:  2011 Jul 
Date Detail:
Created Date:  2011-8-1     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101256510     Medline TA:  Congenit Heart Dis     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
© 2011 Copyright the Authors. Congenital Heart Disease © 2011 Wiley Periodicals, Inc.
Affiliation:
Department of Pediatrics, Division of Pediatric Cardiology Department of Pediatrics Department of Pediatrics, Division of Pediatric Rheumatology, Mayo Clinic College of Medicine, Rochester, Minn, USA.
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