Warfarin
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Skin necrosis: case report A 5-years and 9-months old boy developed skin necrosis of the fingers in left hand while receiving anticoagulant therapy with warfarin. The boy was diagnosed with a congenital heart defect (double-inflow single anatomically left ventricle of the heart and stenosis of the pulmonary artery) immediately after birth. On day 18 day, he underwent surgery for the imposition of a subclavian-pulmonary anastomosis on the left and ligation of the patent ductus arteriosus. At the age of 1 year, he underwent a right-sided bidirectional cavopulmonary anastomosis and ligation of the previously applied anastomosis. At the age of 2 years, he underwent transluminal balloon angioplasty of the left pulmonary artery. At age of 4 years (in 2017), he underwent a Fontan procedure in the modification of the extracardiac conduit and closure of the major aortopulmonary collateral arteries. Additionally, he was started on warfarin 2.5 mg/day [route not stated] with recommendation to regularly monitor his INR; however, the therapy was continued without any monitoring. In December 2018, increasing oedematous syndrome was noted, and a diagnosis of protein-losing enteropathy was made [unrelated to warfarin therapy]. He received treatment with albumin, while the warfarin therapy was continued at the same dose without any monitoring. In Jan 2019, he was diagnosed with microcirculation disorders of the left-hand fingers (II, III and IV), cyanosis of the distal phalanges and stitching pain in the fingers. Further examinations showed increase in preexisting oedema (anasarca) and dyspnoea at rest [unrelated to warfarin therapy]. Therefore, he was hospitalised. His treatment was started with heparin, and some improvement was noted in the microcirculation of finger-II. Thereafter, he was shifted to the cardiology department of another hospital. On day 3 of admission (at the new hospital), his fingers-III and IV were noted as bluish-purple in color. A scab and skin necrosis of the distal phalanx of finger-IV was also noted. He also complained about pain in the fingers when moving and touching objects. Laboratory tests showed the following: hypoglobulinaemia (up to 26 g/L), low iron level 4.7 µmol/L, proBNP level increased (to 449.8), INR was 8, D-dimer 0.27, protein-C level low (27%), proteinuria up to 0.7 g/L and alpha-1 antitrypsin high in the feces. Additionally, polymorphisms in the coagulation genes (ITGB3 and SERPINE1) was noted. A CT scan showed a thrombus in the right internal jugular vein and the major aortopulmonary collaterals. Considering his microcirculation disorders the fingers of the left hand and uncontrolled administration of warfarin before, a diagnosis of warfarin-associated necrosis was confirmed. On day 1 of hospitalisation (at the new hospital), the boy’s warfarin therapy was stopped. His treatment was started with nadroparin-calcium and pentoxifylline. Subsequent improvement was noted in his condition. On day 12 of hospitalisation, he underwent endovascular closure of the major aortopulmonary collate
