Surgery in patients with congenital factor VII deficiency - a single center study.

INTRODUCTION: Congenital factor VII deficiency is a rare hemorrhagic disorder inherited in an autosomal recessive pattern. Surgical treatment with insufficient diathesis correction is burdened with high risk of bleeding complications. The aim of the study was evaluation of the surgical outcome in patients with congenital factor VII deficiency and assessment of the efficacy and safety of recombinant activated factor VII (rFVIIa) used for perioperative hemostatic coverage in our two schemas of substitutive therapy. MATERIAL AND METHODS: In the years 2002-2017 a total of 22 patients with congenital factor VII deficiency were subjected to surgery. Substitution therapy relied on rFVIIa used in two schemas. One involved 15 patients with factor VII activity of<10% of normal value who were injected rFVIIa at a dose of 30 µg/kg b.w. every12 hours on surgery day, 15 µg/kg b.w. every 12 hours on the first postoperative day and 15 µg/kg b.w. every 24 hours on the following days. The second schema involved 7 patients with factor VII activity of 10-25% of normal value who were given rFVIIa at a dose of 15 µg/kg b.w. every 12 hours on surgery day and the first postoperative day; then the same dose was administered every 24 hours on consecutive days. The treatment continued for 4-10 days. RESULTS: In the 22 patients a total of 26 surgeries were performed; 17 surgeries in 15 patients with factor VII<10% of normal and 9 in 7 patients with factor VII deficiency of 10-25% of normal. The surgeries included: 9 cholecystectomies (8 laparoscopic,1 open), 7 thyroidectomy procedures, 2 exploratory laparotomies, 1 left hemicolectomy, 1 total proctocolectomy, 3 inguinal hernia repairs and 3 excisions of varicose veins. One patient with factor VII activity of 9% required an additional dose of rFVIIa in the intraoperative period due to diathesis bleeding. Intraoperative hemostasis was normal for all other patients; no postoperative hemorrhagic complications were reported. In patients with FVII activity<10% average daily dose of rFVIIa was 31.3(range 20-56) µg/kg b.w., total daily dose 186(136-303) µg/kg b.w., total dose of rFVIIa-15.2(12-112) mg. In patients with FVII activity 10-25% the doses were 21.2(15-31), 117(46-271) µg/kg b.w. and 9.1(6-17) mg respectively. CONCLUSIONS: Surgery in patients with congenital factor VII deficiency can be safely and efficiently performed with rFVIIa as substitutive treatment securing perioperative hemostasis.

Prevention of bleeding and hemorrhagic complications in surgical patients with inherited factor VII deficiency.

Inherited factor VII (FVII) deficiency is a rare autosomal recessive hemorrhagic disorder. The major clinical symptoms include: bleeding from the oral cavity, epistaxis, menorrhagia, spontaneous hemarthros, bleeding to the gastrointestinal tract and central nervous system, and perioperative bleeding. The aim of this study was to present our experience in preventing bleeding and hemorrhagic disorders in surgical patients with inherited FVII deficiency by using recombinant activated FVIIa (rFVIIa), and with prothrombin complex concentrates (PCCs). In 2002-2011, 17 patients with inherited FVII deficiency underwent surgery. Thirteen patients had isolated FVII deficiency below 10%, and four patients 10-25. To prevent bleeding and hemorrhagic complications, we administered small single doses of rFVIIa (Novo-Seven) at 12-h intervals to 15 patients on surgery day and on day 1 following surgery, then every 24 h; PCCs were administered (Prothromplex, Beriplex) to two patients. No symptoms of bleeding, hemorrhagic or thromboembolic complications were observed in the perioperative and 1-month observation period in surgical patients treated with rFVIIa. One patient treated with PCC (Prothromplex) developed distal deep vein thrombosis on postoperative day 7. The results suggest that small, single, every 12-h doses of rFVIIa (NovoSeven) and in next days after surgery one time every 24 h are well tolerated and effective for prevention of thromboembolic, bleeding and hemorrhagic complications in FVII-deficient patients. Antithrombotic prophylaxis with low-molecular-weight heparin should be applied in patients using PCCs.

Prevalence rate for inherited thrombophilia in patients with chronic and recurrent venous leg ulceration.

The aim of the study was to determine the prevalence rate for inherited thrombophilia (IT) in patients with chronic (CVU) and recurrent venous leg ulceration. We also investigated and evaluated the severity of the clinical pattern of CVU in patients with and without IT. We examined 110 patients with CVU (the study group) and 110 healthy subjects (the control group). We prepared a questionnaire to be completed by each study participant. Ultrasound Doppler color imaging or/and duplex ultrasonography was performed to evaluate the efficiency of the venous system. The ankle-brachial index was calculated to determine the efficiency of the arterial system. We examined both groups for the presence of IT. IT was diagnosed in 30% of study group and in 1.8% of control group. Our diagnoses of deep vein thrombosis (DVT) were based on medical interviews, physical examinations, and an ultrasonography of the venous system and concerned 64 study group patients (58.2%), 35 of whom (31.8%) experienced recurrent DVT. Proximal and/or distal DVT was determined in an interview and/or by an ultrasonography performed for all patients with CVU and IT. In 94% of these patients, DVT was recurrent, and in 88% of patients with CVU and IT, we observed recurrent DVT and CVU. It recurred more often and persisted longer when compared to patients with CVU and no IT, despite similar management. No differences were observed in ulcer size, localization, or pain level related to ulceration between patients with CVU and IT and those with CVU and no IT.

Perioperative bridging therapy with low molecular weight heparin for patients with inherited thrombophilia and antiphospholipid syndrome on long-term acenokumarol therapy.

The aim of the study is to present our own perioperative bridging therapy with low molecular weight heparin (LMWH) for surgical patients with thrombophilia on long-term acenokumarol therapy [oral anticoagulant (OAC)]. In some European countries, the drug used in secondary antithrombotic prophylaxis is acenokumarol. Forty-two patients with inherited thrombophilia and 21 with antiphospholipid syndrome underwent surgery. All patients were on long-term OAC. This OAC was interrupted 2 days before elective surgery and since that day half of the individual therapeutic dose of LMWH was administered. On day of surgery, the LMWH therapeutic dose was divided into two parts. Starting with day 2 after surgery, the patient was again given half of the individual dose of LMWH every 24 h. On day 4, OAC was additionally included. Both drugs were administered until stabilization of international normalized ratio (INR) values within the therapeutic target for 2 consecutive days. LMWH was then interrupted, whereas OAC continued. No symptoms or episodes of venous thromboembolism were observed. No intraoperative or postoperative hemorrhagic complications were reported. The results suggest that our perioperative bridging therapy is safe and effective for prevention of thromboembolic and hemorrhagic complications.

[Small volume resuscitation for acute intraoperative haemorrhage: comparison of 7.5% saline and 6% hydroxyethyl starch]. / Porównanie wplywu przetoczen malych objetosci 7,5% roztworu NaCL lub 6% roztworu hydroksy-etylowanej skrobi na zaburzenia hemodynamiczne, morfologiczne i elektrolitowe u chorych ze sródoperacyjna, ostra utrata krwi krazacej.

BACKGROUND: The immediate effect of acute haemorrhage is a significant reduction in tissue blood flow, frequently resulting in haemorrhagic shock. The main aim of resuscitation after bleeding is the immediate restoration of intravascular volume. Intravenous administration of volume expanders should be commenced immediately, regardless of whether they are colloids or electrolytes. The purpose of the study was to analyze haemodynamic changes during intraoperative acute bleeding and to compare the effects of intravenous infusion with 7.5% saline solution to 6% starch solution on the volume resuscitation process. METHODS: Sixty adult patients, of both sexes, in whom massive loss of blood followed by rapid cardiovascular collapse occurred during elective surgery, were enrolled in the study. In addition to standard fluid transfusion, all patients received either 4 mL kg(-1) of 7.5% NaCl solution or an equal volume of 6% of hydroxyethyl starch. Heart rate (HR) and mean arterial pressure (MAP) were noted. Cardiac output (CO) and systemic vascular resistance (SVR) were measured using a descending aortic blood flow ultrasound monitor. RESULTS: Massive intraoperative haemorrhage resulted in a rapid decrease in CO and SV with a simultaneous increase in HR and SVR in all patients. Injection of hypertonic salt or 6% HAES over 5 min increased the CO and SV. HR and SVR returned quickly to pre-existing levels and remained so until the end of the procedures. CONCLUSION: Small volume resuscitation with 7.5% saline or starch can be regarded as an efficient and effective method for restoring intravascular volume.