Idiopathic infantile arterial calcification: Two case reports, a review of the literature and a role for cardiac transplantation
Andrew C. Glatz
Division of Cardiology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
Search for more papers by this authorBruce R. Pawel
Department of Pathology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
Search for more papers by this authorDaphne T. Hsu
Department of Clinical Pediatrics, Columbia University Medical Center, New York, NY, USA
Search for more papers by this authorPaul Weinberg
Division of Cardiology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
Department of Pathology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
Search for more papers by this authorMaryanne R. K. Chrisant
Division of Cardiology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
Search for more papers by this authorAndrew C. Glatz
Division of Cardiology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
Search for more papers by this authorBruce R. Pawel
Department of Pathology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
Search for more papers by this authorDaphne T. Hsu
Department of Clinical Pediatrics, Columbia University Medical Center, New York, NY, USA
Search for more papers by this authorPaul Weinberg
Division of Cardiology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
Department of Pathology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
Search for more papers by this authorMaryanne R. K. Chrisant
Division of Cardiology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
Search for more papers by this authorAbstract
Abstract: Idiopathic infantile arterial calcification (IIAC) is a rare, but important, cause of rapidly progressive ischemic heart disease in children. In this paper, we report two recent cases of IIAC seen at tertiary referral hospitals. Both cases presented in infancy with signs of heart failure and, ultimately, died with the diagnosis of IIAC confirmed at postmortem examination. A thorough review of the literature reveals approximately 160 reported cases of IIAC. The clinical outcomes, radiographic findings and pathologic details are summarized. Proposed etiologic mechanisms are reviewed, including promising research into the role of inorganic pyrophosphate as a regulatory factor in the development of IIAC. Because of the typically fatal outcome of IIAC and the lack of proven therapies, the potential role for cardiac transplantation is discussed.
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