Cardiac surgery in patients with haemophilia

Abstract

Today the populations of haemophilia patients in many countries have a higher life expectancy than previously known, and age-related disorders such as arterial disease are expected to become more prevalent, calling for surgical intervention. Cardiac surgery constitutes a major haemostatic challenge because of sternotomy, the need of total heparinization, extracorporal circulation, mild hypothermia and cardiac arrest. To evaluate our current experience and results with cardiac surgery in patients with haemophilia the present case series report on six patients with haemophilia A (Severe = 1, Moderate = 1, Mild = 4) undergoing cardiac surgery (coronary artery bypass grafting; CABG = 2, aortic valve replacement = 1, CABG + aortic valve replacement = 2, ventricular resection + mitral valve reconstruction = 1). The present paper provides detailed information on the haemostatic treatment regimens adopted (factor concentrate dosages, timing and duration) and postoperative thromboprophylaxis (dosing and duration of low molecular weight heparin). Moreover, we present data on concomitant disorders (hypertension, hypercholesterolaemia, atrial fibrillation and diabetes), left ventricle ejection fraction (30-60%), type of anaesthesia, total amount of heparin (34 500-53 500 IU) and duration of extracorporeal circulation (80-115 min). Clinical outcomes included: re-operation because of bleeding (none), transfusion requirements, peri- and postoperative blood loss and complications and postoperative development of inhibitors (none). Clinical outcomes were compared with a control group of patients (n = 5993) without haemophilia and we found no difference in postoperative morbidity. Adopting meticulously supervised haemostatic treatment regimens, we have successfully performed major cardiac surgery in patients with haemophilia A. The clinical outcome as well as the severity and incidence of postoperative complications were similar to patients without haemophilia.

How I treat age-related morbidities in elderly persons with hemophilia

“…Continuous infusion of CFC before, during, and after surgery until wound healing is sufficiently advanced should be preferred over bolus infusion. 56,57 For personal use only. on April 2, 2019. by guest www.bloodjournal.org From Despite the use of these strategies of replacement therapy, the aforementioned revascularization procedures and the associated antithrombotic regimens sometimes cause excessive bleeding, which may be difficult to control and increase mortality.…”

Successful double bypass in a patient with severe hemophilia A: a case report

“…Today the life expectancy in the hemophilia people is similar to the non-haemophilics and age-related cardiovascular disorders to become more prevalent [1][2][3]. Cardiac surgeries are considered a very high risk for patient with hemophilia because of the need of heparinization and extracorporeal circulation [1]. Continuous infusion of clotting factors associated with tranexamic acid that increases clot resistance [4], permits these interventions, but only few centres around the world have experience to performing cardiac surgery in haemophilic patients [1].…”

“…For several years it was believed that patients with hemophilia were protected against these diseases, but although some studies showed the low mortality for coronary vessel diseases in haemophilic patients, it was also shown that the reduced level of FVIII in hemophilia A people can not protect against heart disease. Today the life expectancy in the hemophilia people is similar to the non-haemophilics and age-related cardiovascular disorders to become more prevalent [1][2][3]. Cardiac surgeries are considered a very high risk for patient with hemophilia because of the need of heparinization and extracorporeal circulation [1].…”

The Aging Patient with Hemophilia: Complications, Comorbidities, and Management Issues

“…Postoperative morbidity was reported to be similar to patients without hemophilia. 43 Some have suggested using continuous infusion (off-label) recombinant factor VIII or IX over bolus factor infusion perioperatively to avoid peaks of factor VIII or IX activity, to maintain stable factor levels, and to reduce thrombosis risk. 29,40 Because of the bleeding risks in patients with hemophilia, cardiac bypass surgery may be preferred over percutaneous coronary intervention with stenting given the requirement for dual antiplatelet agents for extended periods of time, the risk of re-stenosis, and the potential need for repeated procedures.…”