Left ventricular assist device and heart transplantation in hemophilia a patient.
ABSTRACT We report here a hemophilia patient who was bridged with a left ventricle assist device and later received heart transplantation. Preparation for surgery with factor VIII supplementation, intraoperative conduct of surgery, and challenges of postoperative course are described with a brief literature review.
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ABSTRACT: A best evidence topic in cardiac surgery was written according to a structured protocol. The question addressed was whether patients with haemophilia undergoing cardiac surgery have good surgical outcomes. Haemophilia A and haemophilia B are sex-linked recessive inherited diseases affecting males only, with females acting as carriers. The conditions result in various degrees of factor VIII or factor IX deficiency, respectively. The life expectancy of haemophilia patients is increasing and now approaches that of the general male population, and they are confronted with age-related co-morbidity, including ischaemic cardiovascular disease. Replacement of the deficient factor (VIII for haemophilia A and IX for haemophilia B) is the cornerstone of treatment; other therapeutic options include tranexamic acid, desmopressin and aprotinin. Recently, the advent of recombinant factor VIII and IX has eliminated the infective risk of using factor concentrates, such as prothrombin complex concentrate or fresh frozen plasma. A total of 84 papers were found using the reported search criteria, and out of this 25 papers, selected with reference to a more modern date range, provided the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results were tabulated. We conclude that there is lack of good-quality evidence and that, in all probability, these papers are subject to publication bias as poor outcomes are unlikely to have been reported. However, all the reported series showed that good outcomes are possible in this specific subgroup of patients given the correct approach. The data accrued from these studies (a total of 30 adults and three children) suggest that routine cardiac surgery can be performed safely in patients with haemophilia, with minimal morbidity and mortality. We identified the following key points to achieve this result: a team approach, a factor replacement protocol and perioperative monitoring of factor levels. Intraoperative plasma factor levels can be easily measured before heparin and after protamine sulphate administration, whereas during cardiopulmonary bypass this will require a chromogenic method. Exposure to factor concentrates early in the life might predispose a patient with severe haemophilia to the development of inhibitors. Moreover, the absence of inhibitors should be confirmed before any surgical procedure.Interactive Cardiovascular and Thoracic Surgery 06/2011; 13(3):320-31. · 1.11 Impact Factor
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ABSTRACT: To report the management of hemophilia in a patient with dilated cardiomyopathy during application of the Berlin-Heart biventricular assist. Case report. The Berlin-Heart biventricular assist was successfully used as a bridge to heart transplantation (178 days) in a child with hemophilia A; post-transplant extracorporeal membrane oxygenation was implanted until the patient recovered from primary graft failure. Anticoagulant therapy varied as a function of patient status: 1) postoperative bleeding was treated by partial replacement of deficient factors (VII and VIII) and hemoderivatives in order to maintain factor VIII at 50% of normal levels; 2) once the bleeding had stopped, the effect of the hemophilia itself maintained the degree of anticoagulation required by the ventricular assist device; and 3) transplant surgery was followed by complete replacement of factor VIII and intravenous heparinization (a simple way of preventing clot formation in the device and to ensure proper scarring of surgical wounds). Selection of anticoagulant therapy as a function of patient status in terms of bleeding and surgical-wound scarring progress is vital for the proper functioning of support techniques (Berlin-Heart biventricular assist and extracorporeal membrane oxygenation) in hemophiliac patients. Collagen dressings placed on surgical wounds achieved good functional and aesthetic results, as well as mechanically isolating the scars from the exterior.Pediatric Critical Care Medicine 09/2011; 12(6):e432-5. · 2.33 Impact Factor
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ABSTRACT: The present study summarizes the results of 12 cardiac surgical procedures performed in a carrier of Haemophilia B and in six patients with Haemophilia A at a single centre from 1979 to 1998. The median age of the patients at the time of intervention was 56 years ranging from 18 years to 73 years. The six patients with Haemophilia A ranged in severity from moderately to mildly affected. Three patients were hepatitis C antibody positive. No patients were HIV antibody or hepatitis B surface antigen positive. The cardiac procedures included cardiac catheterization (n=4), coronary artery bypass surgery (n=2), percutaneous transluminal coronary angioplasty (n=1), cardiac valve replacement (AVR n=1 and AVR/MVR n=2), and closure of an atrial septal defect and subsequent drainage of a pericardial effusion (n=1). No patients had demonstrable inhibitors at the time of surgery. Haemostasis was achieved with AHF in 10/11 procedures and high purity factor IX (Immunine) in one procedure. The initial procedures involved intermittent bolus factor therapy while more recently, AHF was administered by continuous intravenous infusion. All patients demonstrated excellent intra- and post-operative haemostasis. These results, although from a small and varied group of patients, demonstrate that cardiac surgical procedures can be performed safely in patients with Haemophilia.Haemophilia 04/2000; 6(2):84-8. · 2.47 Impact Factor