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The difficulty in diagnosing idiopathic arterial calcification of infancy, its variation in presentation, and the importance of autopsy

Published online by Cambridge University Press:  01 December 2008

Kathrin Hault ,
Neil J. Sebire ,
Siew Y. Ho  and
Mary N. Sheppard
Kathrin Hault
Affiliation:
CRY Centre for Cardiac Pathology, National Heart and Lung Institute, Royal Brompton Campus, London, England
Neil J. Sebire
Affiliation:
Department of Histopathology, Great Ormond Street Hospital for Children, London, England
Siew Y. Ho
Affiliation:
Department of Cardiac Morphology, National Heart and Lung Institute, London, England
Mary N. Sheppard*
Affiliation:
CRY Centre for Cardiac Pathology, National Heart and Lung Institute, Royal Brompton Campus, London, England
*
Correspondence to: Dr Mary N. Sheppard, Consultant Histopathologist, CRY Centre for Cardiac Pathology, Royal Brompton Hospital, Department of Histopathology, Sydney Street, London, SW3 6NP, United Kingdom. Tel: +44 207 351 8424; Fax: +44 207 351 8293; E-mail: m.sheppard@rbht.nhs.uk
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Abstract

Idiopathic calcification of the coronary arteries is a rare hereditary condition of infancy. Complications include cardiac ischaemia, cardiac failure, and systemic hypertension. We present three patients with this condition, which in each case masqueraded as other cardiac diseases, with no indication of the specific diagnosis prior to autopsy.

A 5 month old female presented with respiratory failure and hypertension and died within 24 hours of admission. All the coronary arteries were thick-walled, with narrow lumens. The aorta, great vessels, and renal arteries also showed thickening of the wall. Histology confirmed calcium in the internal elastic lamina of all vessels. The second patient was a female baby of 2 months, diagnosed with a large ventricular septal defect. She died suddenly prior to surgery. At autopsy, the orifice of the right coronary artery was reduced to a pinhole. The coronary arteries showed white patches of calcification, with associated ventricular infarction. The third patient was an 11 year old female who presented with cardiac failure, and had been diagnosed with dilated cardiomyopathy. Two weeks later, she died suddenly. The coronary arteries were patent, but firm with calcification and narrowed, with associated ventricular infarction. Our experience shows that idiopathic arterial calcification of infancy should always be considered in infants and children presenting with hypertension, cardiac failure, or sudden death.

Keywords

Coronary arteryinfantmyocardial infarctionrenal arteryhypertensionsudden death
Type
Original Article
Information
Cardiology in the Young , Volume 18 , Issue 6 , December 2008 , pp. 624 - 627
Copyright
Copyright © Cambridge University Press 2008

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