Idiopathic infantile arterial calcification: two case reports, a review of the literature and a role for cardiac transplantation.
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.
Division of Cardiology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA. glatz@email.chop.edu
Andrew C
AC
Glatz
Bruce R
BR
Pawel
Daphne T
DT
Hsu
Paul
P
Weinberg
Maryanne R K
MR
Chrisant
D001158:Arteries / Q000473:pathology*; D001485:Basement Membrane / Q000473:pathology; D002114:Calcinosis / Q000473:pathology* / Q000601:surgery; D009202:Cardiomyopathies / Q000473:pathology; D003331:Coronary Vessels / Q000473:pathology; D011756:Diphosphates / Q000627:therapeutic use; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D016027:Heart Transplantation; D006801:Humans; D007223:Infant; D017202:Myocardial Ischemia / Q000209:etiology / Q000473:pathology* / Q000601:surgery; D009206:Myocardium / Q000473:pathology; D017539:Tunica Intima / Q000473:pathology; D014652:Vascular Diseases
D011756:Diphosphates