A rare cause of recurrent modified Blalock-Taussig shunt thrombosis: Antiphospholipid antibodies

Role of thrombophilia factors in acute systemic–pulmonary shunt obstruction

“…Thrombophilia was detected in three of eight patients (38%) who underwent intervention due to shunt thrombosis. Factor V Leiden heterozygous mutation was detected in one patient, anti‐phospholipid anybody was positive in another, and the third patient had heterozygous protein C deficiency (34% of the normal level of functioning protein C).…”

“…Acute systemic shunt thrombosis in patients with systemic–pulmonary shunt cannot be completely explained by hypoxia, cyanosis, polycythemia, and the foreign body used for the shunt . While mechanical/hemodynamic factors such as systemic hypotension, pulmonary hypertension, external pressure, and shunt distortion may promote thrombosis, particularly in the postoperative period, in the literature there are reports of acute shunt thrombosis associated with thrombophilia disorders such as factor V Leiden mutation, anti‐phospholipid syndrome, and protein C deficiency . A common proposal is that in patients with unexplained shunt thrombosis, inherited and acquired thrombophilia factors should be investigated to clarify its etiology .…”

“…Although many strategies are used to treat systemic–pulmonary shunt thrombosis, including treatment with acetyl salicylic acid, heparin, and warfarin, the role of inherited and acquired thrombophilia factors has not been thoroughly investigated. In the literature, acute shunt thrombosis related to protein C deficiency and anti‐phospholipid syndrome have been reported, yet there is no study on the role of thrombophilia factors in patients with acute shunt thrombosis, as far as we know. In the present prospective study, patients who developed acute shunt thrombosis were examined in terms of inherited or acquired thrombophilia factors.…”