Cardiac surgery in patients with Hemophilia:is it safe?

Abstract

Background: The life expectancy of hemophiliacs is similar to that of the general population. As a result, the prevalence of age-related cardiovascular diseases has increased. We present our experience with hemophilia patients who underwent cardiac surgery in our Medical Center between 2004 and 2019. Methods: All hemophilia patients who underwent cardiac surgery were identified, and their peri-operative data evaluated retrospectively. Results: Ten patients were identified: six with hemophilia-A, one with hemophilia-B, and three with hemophilia-C (factor XI deficiency). Cardiac procedures included ten coronary artery bypass grafts and one aortic valve replacement. Hemophilia-A and B patients were treated with factor substitution, whereas patients with factor XI deficiency were treated with fresh frozen plasma. One patient died, and one patient suffered from non-active gastrointestinal bleeding. Conclusions: While major cardiac surgery can be performed safely on patients with hemophilia, a multidisciplinary team approach and strict postoperative monitoring are essential in order to achieve optimal results.

Spinal Subdural Hematoma in a Young Adult with Hemophilia A: A Case Report

“…Our patient's coagulative function was so poor that the operation was thought to be too risky after consultation with neurologist [18]. Therefore, tranexamic acid was given to stop bleeding and polyene phosphatidylcholine for the trophic nerve.…”

Cardiac Surgery in Patients With Blood Disorders

“…Desired cardiac surgery factor levels, including the use of cardiopulmonary bypass (CPB), are not specified by the WHF. The studies' target levels were set at a factor level more than 80% of normal, reported using the activated partial thromboplastin time (aPTT) [19][20][21][22][23] or aPTT in combination with the more recent TEG R-time [24][25][26], to confirm effectiveness of factor replacement. The targets were achieved using bolus doses, most studies reported either use of recombinant (rFVIII/rFIX) or plasma derived (pdFVIII/ pdFIX) factor concentrate [19,20,22,24,25]; the WHF does not state a preference between the two [18].…”

“…At CPB discontinuation several studies reported autologous blood transfusion using cell saver [19,20,[24][25][26].…”

How to manage bleeding disorders in aging patients needing surgery

“…2 The lit er a ture sug gests that despite the major hemo static chal lenges of var i ous car diac inter ven tions, care ful, mul ti disci plin ary plan ning can help achieve opti mal out comes with min i mal mor bid ity and mor tal ity in patients with bleed ing disor ders. 53,54,[56][57][58][59][60] However, these data may be sub ject to poten tial pub li ca tion bias, and older adult patients are under rep re sented. †Dysfibrinogenemia often pres ents with a throm botic phe no type.…”

“…61 The lack of FXI con cen trates in the United States neces si tates the use of FFP infu sions (usu ally 10-20 mL/kg) to replace FXI perioperatively or the use of bypass agents or antifibrinolytic agents. 59,[61][62][63] In older adult patients, large vol umes of FFP are a major risk factor for cir cu la tory over load, espe cially in those with valve dis ease or con ges tive heart fail ure. In such patients, major sur gery would require thor ough fac tor replace ment and large vol umes of plasma, which may pose a substantial risk.…”