Thrombotic occlusion of Blalock-Taussig shunt in a patient with unnoticed protein C deficiency

Watanabe, Manabu; Aoki, Marcelo Saldanha; Fujiwara, Tadashi

doi:10.1007/s11748-008-0295-y

Cited by 7 publications

(5 citation statements)

References 5 publications

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“…Rare coagulopathies, such as protein C deficiency [23], and primary antiphospholipid syndrome [24] have also been reported to cause shunt thrombosis.…”

Section: Anticoagulation-coagulopathymentioning

confidence: 99%

Modified Blalock Taussig shunt: a not-so-simple palliative procedure

Dirks

Prêtre²,

Knirsch

et al. 2013

European Journal of Cardio-Thoracic Surgery

107

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OBJECTIVES: Thirty-two consecutive isolated modified Blalock Taussig (BT) shunts performed in infancy since 2004 were reviewed and analysed to identify the risk factors for shunt intervention and mortality. METHODS:Sternotomy was the only approach used. Median age and weight were 10.5 (range 1-74) days and 2.9 (1.9-4.4) kg, respectively. Shunt palliation was performed for biventricular hearts (Tetralogy of Fallot/double outlet right ventricle/transposition of great arteries_ventricular septal defect_pulmonary stenosis/pulmonary atresia_ventricular septal defect/others) in 21, and univentricular hearts in 11, patients. Hypoplastic left heart syndrome patients were excluded. Two procedures required cardiopulmonary bypass. Median shunt size was 3.5 (3-4) mm and median shunt size/kg body weight was 1.2 (0.9-1.7) mm/kg. Reduction in shunt size was necessary in 5 of 32 (16%) patients. RESULTS:Three of 32 (9%) patients died after 3 (1-15) days due to cardiorespiratory decompensation. Lower body weight (P = 0.04) and bigger shunt size/kg of body weight (P = 0.004) were significant risk factors for mortality. Acute shunt thrombosis was observed in 3 of 32 (9%), none leading to death. Need for cardiac decongestive therapy was associated with univentricular hearts (P < 0.001), bigger shunt size (P = 0.054) and longer hospital stay (P = 0.005). Twenty-eight patients have undergone a successful shunt takedown at a median age of 5.5 (0.5-11.9) months, without late mortality.CONCLUSIONS: Palliation with a modified BT shunt continues to be indicated despite increased thrust on primary corrective surgery. Though seemingly simple, it is associated with significant morbidity and mortality. Effective over-shunting and acute shunt thrombosis are the lingering problems of shunt therapy.

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“…Rare coagulopathies, such as protein C deficiency [23], and primary antiphospholipid syndrome [24] have also been reported to cause shunt thrombosis.…”

Section: Anticoagulation-coagulopathymentioning

confidence: 99%

Modified Blalock Taussig shunt: a not-so-simple palliative procedure

Dirks

Prêtre²,

Knirsch

et al. 2013

European Journal of Cardio-Thoracic Surgery

107

View full text Add to dashboard Cite

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“…In the literature, patients with protein C deficiency who were diagnosed with shunt thrombosis and had anti-phospholipid syndrome, were able to be successfully maintained with warfarin therapy. 9,20 We have also ensured that the present patients with similar diagnoses without complications are also having aspirin and warfarin concomitantly.…”

Section: Discussionmentioning

confidence: 85%

Role of thrombophilia factors in acute systemic–pulmonary shunt obstruction

Ergül

Kiplapinar

Öztürk

et al. 2015

Pediatrics International

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“…Certain thromboembolic factors such as protein C deficiency, factor V Leiden or protrombin 20210 mutation have been reported with cardiac thrombosis in children (8,9). Deally et al (10) published a newborn with mBT shunt thrombosis caused by APS in 1999.…”

Section: Discussionmentioning

confidence: 99%