Off-Pump Myocardial Revascularization in a Diabetic Patient with Severe Hemophilia B and Impaired Left Ventricular Function: Hematological and Operative Strategies

Grandmougin, Daniel; Delolme, Maria-Christina; Reynaud, J.; Barral, Xavier

doi:10.1111/j.1540-8191.2005.2004108.x

Cited by 11 publications

(24 citation statements)

References 10 publications

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“…Grandmougin et al [9] administered an intraoperative bolus of aspirin 250 mg to prevent graft thrombosis, as well as infusions of rfIX, in off-pump myocardial revascularisation [off-pump coronary artery bypass (OPCAB)] of a patient with haemophilia B.…”

Section: Resultsmentioning

confidence: 99%

Do patients with haemophilia undergoing cardiac surgery have good surgical outcomes?

Rossi

Jayaram

Sayeed

2011

Interactive CardioVascular and Thoracic Surgery

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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.

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Section: Resultsmentioning

confidence: 99%

Do patients with haemophilia undergoing cardiac surgery have good surgical outcomes?

Rossi

Jayaram

Sayeed

2011

Interactive CardioVascular and Thoracic Surgery

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“…In general, outcomes across reported cases and series were exceptionally good, considering the high‐risk nature of cardiac surgery even among patients without bleeding diatheses. One group opted for off‐pump coronary artery bypass grafting (CABG), reasoning that off‐pump surgery would cause less coagulopathy than traditional cardiopulmonary bypass [29]. One haemophilia B carrier underwent atrial septal defect closure with Immunine by bolus dosing over 11 days but required drainage of a pericardial effusion later [11].…”

Section: Discussion and Review Of The Literaturementioning

confidence: 99%

“…Some using bolus infusions of FIX with target levels in the lower limit of the normal range (∼0.50–0.60 IU mL −1 ) experienced bleeding complications [26]. Only one previous case report describes the maintenance of near 1.00 IU mL −1 by CI of plasma‐derived FIX [29], requiring a daily dose of 450 IU h −1 infusion of plasma‐derived FIX. Although the patient’s weight is not given, the dosage of FIX appears comparable to our patient’s requirements.…”

Section: Discussion and Review Of The Literaturementioning

confidence: 99%

“…There are several case reports and case series of percutaneous coronary stent insertion and of cardiac surgery in patients with haemophilia A [8][9][10][11][12][13][14] and haemophilia B [11,[15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30]. Some of the older reports describe perioperative haemostatic strategies that are now outdated, such as plasmapheresis with fresh frozen plasma replacement [17] and reliance on prothrombin complex concentrate [16,18,21].…”

Section: Discussion and Review Of The Literaturementioning

confidence: 99%

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Cardiac surgery in patients with haemophilia B: a case report and review of the literature

et al. 2009

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Cardiac surgery for coronary heart disease, and for calcific and degenerative valvular heart disease, will likely become more frequent with an ageing haemophilia population. Our report describes the successful management of an individual with mild haemophilia B undergoing elective aortic valve replacement using a continuous infusion of recombinant factor IX. Emphasis is placed on the multidisciplinary coordination of care required across three hospital sites to ensure an uncomplicated peri- and postoperative course. We also provide a review of the current literature on cardiac surgery in patients with haemophilia B.

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“…The reported complications consisted of post‐pericardiotomy syndrome, hemopericardium and tamponade, aortic dissection, atrial fibrillation, gastrointestinal bleeding, and hemarthrosis . Off‐pump CABG was performed only in three patients with severe hemophilia . Two cases were uneventful and one case was complicated with early vein‐graft occlusion (Table ) …”

Section: Review Of the Literaturementioning

confidence: 99%

Replacement therapy for coronary artery bypass surgery in patients with hemophilia A and B

Kanellopoulou

Nomikou

2018

J Card Surg

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There are only a few cases of patients with hemophilia A and B who have undergone coronary artery bypass surgery. High levels of replacement therapy with factor concentrate either with bolus or continuous infusion are usually required pre-operatively and during the first post-operative days in order to maintain the coagulation deficient factor levels greater than 80% of normal. Heparinization during cardiopulmonary bypass appears to be safe and intra-operative blood salvage using cell saver techniques reduce the need for transfusions.

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Off-Pump Myocardial Revascularization in a Diabetic Patient with Severe Hemophilia B and Impaired Left Ventricular Function: Hematological and Operative Strategies

Cited by 11 publications

References 10 publications

Do patients with haemophilia undergoing cardiac surgery have good surgical outcomes?

Do patients with haemophilia undergoing cardiac surgery have good surgical outcomes?

Cardiac surgery in patients with haemophilia B: a case report and review of the literature

Replacement therapy for coronary artery bypass surgery in patients with hemophilia A and B

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