A 41-year-old woman, the mother of 3 offspring, with likely heterozygous familial
hypercholesterolemia, had been asymptomatic until age 38 when angina pectoris and
exertional dyspnea appeared leading to the discovery of severe multivessel coronary
artery disease and a massively calcified ascending aorta. Coronary bypass grafting
using the right and left internal mammary arteries did not alleviate the symptoms.
Evidence of overt heart failure subsequently appeared and that led to heart transplantation
at age 41. She died 22 days later. The occurrence of massive diffuse calcification
of the ascending aorta and minimal focal calcification of the abdominal aorta is rare
and in the patient described it appears to be the consequence of heterozygous familial
hypercholesterolemia.
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References
- Cardiovascular features of homozygous familial hypercholesterolemia: analysis of 16 patients.Am J Cardiol. 1984; 54: 20-30
- Porcelain aorta. A comprehensive review.Circulation. 2015; 131: 827-836
- Surgical strategies for severe calcifications of the aorta (porcelain aorta) in two patients with homozygous familial hypercholesterolemia.Am J Cardiol. 2007; 23: 1159-1161
Article info
Publication history
Published online: January 28, 2016
Accepted:
January 22,
2016
Received in revised form:
January 22,
2016
Received:
December 18,
2015
Footnotes
This study was funded by the Baylor Health Care System Foundation through the Cardiovascular Research Review Committee and in cooperation with the Baylor Heart and Vascular Institute.
See page 1385 for disclosure information.
Identification
Copyright
© 2016 Elsevier Inc. Published by Elsevier Inc. All rights reserved.