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Guide Wire Assisted Catheterization and Colored Dye Injection for Vascular Mapping of Monochorionic Twin Placentas
Published on: September 5, 2011
Monoamniotic monochorionic twins discordant for noncompaction cardiomyopathy
Dianna Ng1, Yosr Bouhlal, Philip C Ursell
1Department of Pathology, University of California San Francisco, San Francisco, California 94143-0793, USA.
American Journal of Medical Genetics. Part A
|May 3, 2013
Summary
Monozygotic twins can show different conditions, like cardiac noncompaction, even with shared genetics. This suggests environmental factors influence fetal development and heart conditions.
Area of Science:
- Developmental Biology
- Cardiovascular Science
- Genetics
Background:
- Identical twins can exhibit phenotypic differences, raising questions about zygosity and developmental influences.
- Monochorionic-monoamniotic twins, one with cardiac noncompaction, were observed, prompting investigation into ventricular development.
Observation:
- A case of monochorionic-monoamniotic twin pregnancy identified due to increased nuchal translucency in one fetus.
- One twin presented with isolated cardiac abnormality, including noncompaction cardiomyopathy and complete heart block, and did not survive.
- The co-twin exhibited normal cardiac development and function both prenatally and postnatally.
Findings:
- Pathologic analysis revealed left ventricular noncompaction and aortic/pulmonary valve dysplasia in the affected twin.
- Atrioventricular bundle fibrosis was identified in the cardiac conduction system of the affected twin.
- STR marker analysis confirmed monozygosity despite phenotypic discordance for cardiac noncompaction.
Implications:
- Monozygotic monochorionic-monoamniotic twins can be discordant for congenital heart defects like cardiac noncompaction.
- Findings suggest that factors beyond genetics, potentially including early muscular contraction and blood flow, play a critical role in ventricular morphogenesis.
- Further research is warranted to explore the roles of fibrosis, contractility, and hemodynamics in fetal cardiac development and congenital heart disease.
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