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Review

Left-Right Asymmetry and Human Heterotaxy Syndrome

Isao Shiraishi  1
Toshio Nakanishi  1 , Roger R. Markwald  2 , H.Scott Baldwin  3 , Bradley B. Keller  4 , Deepak Srivastava  5 , Hiroyuki Yamagishi  6
, editors.
In: Etiology and Morphogenesis of Congenital Heart Disease: From Gene Function and Cellular Interaction to Morphology [Internet]. Tokyo: Springer; 2016. Chapter 6.
.
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Review

Left-Right Asymmetry and Human Heterotaxy Syndrome

Isao Shiraishi.
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Excerpt

Heterotaxy syndrome is characterized by a wide variety of cardiac and extra-cardiac congenital malformations that are primarily induced by disorders of the left-right axis determination during early embryonic development. Prognosis of the disease remains unsatisfactory because the syndrome is often associated with complicated congenital heart diseases. Long-term follow-up of heterotaxy patients, particularly those who underwent Fontan procedure, is now one of the most important issues in pediatric and adult congenital heart disease clinics. Collaborative studies between pediatric cardiologists and basic scientists are essential for improving the prognosis of heterotaxy syndrome.

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References

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