How to manage a congenital heart defect in a patient with thrombocytopenia-absent radius syndrome?

TAR syndrome and heart defect

Authors

Keywords:

Thrombocytopenia-absent radius syndrome, TAR syndrome, ventricular septal defect, infant

Abstract

Ventricular septal defect (VSD) can be repaired using cardiopulmonary bypass, resulting in a favorable postoperative outcome with minimal bleeding. Thrombocytopenia-absent radius (TAR) syndrome is rare, occurring in approximately 0.42 out of 100,000 live births. This syndrome is characterized by hypo-megakaryocytic thrombocytopenia and bilateral absent radii. TAR syndrome can be life-threatening within the first 14 months of life due to severe bleeding. In this report, we present the case of a 4-month-old male patient diagnosed with both VSD and TAR syndrome. We describe the surgical management of the VSD as well as the perioperative treatment for hemorrhagic diathesis.

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Author Biography

Buğra Harmandar, Department of Cardiovascular Surgery, Muğla Sıtkı Koçman University Medical Faculty, Muğla, Turkey

Muğla Sıtkı Koçman University Medical Faculty, Department of Cardiovascular Surgery

References

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Kumar C, Sharma D, Pandita A, Bhalerao S. Thrombocytopenia absent radius syndrome with tetralogy of Fallot: a rare association. Int Med Case Rep J. 2015;8:81-5. DOI: https://doi.org/10.2147/IMCRJ.S81770

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Published

2024-04-20

Issue

Section

Case Report

How to Cite

1.
İştar H, Harmandar B. How to manage a congenital heart defect in a patient with thrombocytopenia-absent radius syndrome? TAR syndrome and heart defect. J Surg Med [Internet]. 2024 Apr. 20 [cited 2024 May 18];8(4):78-80. Available from: https://jsurgmed.com/article/view/7580