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

Hande  İştar; Buğra Harmandar

doi:10.28982/josam.7580

Authors

Hande İştar Department of Cardiovascular Surgery, Muğla Sıtkı Koçman University Medical Faculty, Muğla, Turkey https://orcid.org/0000-0002-7150-0171
Buğra Harmandar Department of Cardiovascular Surgery, Muğla Sıtkı Koçman University Medical Faculty, Muğla, Turkey https://orcid.org/0000-0002-7487-1779

DOI:

https://doi.org/10.28982/josam.7580

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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