Why the study?
Neonatal Marfan syndrome has a poor prognosis from severe atrioventricular valve insufficiency, but tricuspid valve replacement is rarely performed in early infancy.
Early multiple-valve replacement (mitral and tricuspid) can be successfully performed in infants with neonatal Marfan syndrome.
Hypothesis-generating for early dual-valve replacement in neonatal Marfan syndrome; prospective studies needed before practice change.
Neonatal Marfan syndrome is a rare condition with poor prognosis because of severe mitral and/or tricuspid valve insufficiency. Mitral valve replacement is sometimes required in early infancy, while tricuspid valve replacement is rarely done. We report the first infant neonatal Marfan syndrome case with a missense variant of c.3706T>C in the fibrillin-1 gene that was successfully managed by mitral and tricuspid valve replacement. Early multiple-valve replacement may sometimes be required during infant age in this genetic syndrome.
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Kawamura et al. (2021) studied this question.
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