A 3-week-old Amish boy with a homozygous MYBPC3 mutation causing severe cardiomyopathy
admitted for refractory heart failure was referred for heart transplant evaluation.
Due to the known risk of early mortality with this mutation
1
including a prior loss of another child in the family with the same phenotype, the
infant was admitted to the hospital for management of heart failure and expedited
evaluation. The patient was stabilized on intravenous milrinone. Labs were remarkable
for high levels of Human Leukocyte Antigen (HLA) antibodies (Panel of Reactive Antibodies
(PRA) Class I: 74%, Class II: 80%) with no known sensitizing events. Maternal history
of multiple miscarriages raised the question whether his HLA antibodies may have resulted
from maternal HLA sensitization with transplacental antibody transfer vs. active antibody
production by the child
2
. Subsequent antibody testing of mother's blood and breast milk revealed nearly identical
HLA antibody profiles to the patient. Due to the unclear role of antibodies transferred
in the breastmilk and ongoing sensitization of the infant, it was recommended that
the mother not breastfeed.Abbreviations:
HLA (Human Leukocyte Antigens), PRA (Panel of Reactive Antibodies)To read this article in full you will need to make a payment
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References
- Homozygous mutation of MYBPC3 associated with severe infantile hypertrophic cardiomyopathy at high frequency among the Amish.Heart. 2008; 94: 1326-1330
- HLA antibody repertoire in infants suggests selectivity in transplacental crossing.Am J Reprod Immunol. 2020; 84: e13264
- Double Volume Exchange Transfusion: A Review of the “Ins and Outs”.NeoReviews. 2013; 14: e513-e520
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