Aortic and Mitral Valve Replacement in a Patient with Hemophilia B

Thankachen, Roy; George, Biju; Shukla, Vinayak; Korula, Roy John

doi:10.1177/021849230701500618

Cited by 7 publications

(19 citation statements)

References 6 publications

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“…Thankachen et al [8] presented a 25-year-old man with haemophilia B who underwent successful mechanical mitral Best Evidence Topic valve replacement (MVR)+AVR under cover of factor IX concentrate. On POD 4, the patient developed cardiac tamponade requiring surgical drainage.…”

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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“…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%

“…Although there may be a risk of thrombosis at supranormal FIX levels [7,35], given the risk of bleeding and the difficulty of organizing re-operation, for our patient we elected to aim for target levels of 1.00 IU mL )1 . As there are only three reports of the use of plasma-derived FIX infusions in cardiac surgery, it is difficult to compare the total unitage of FIX used in our case to these single cases of patients with haemophilia of differing weight and baseline FIX levels [24,29,30]. Previous groups using CI have aimed for lower target FIX levels [24,30], often significantly lower than our target of 1.00 IU mL )1 , and consequently had lower FIX requirements.…”

Section: Discussion and Review Of The Literaturementioning

confidence: 99%

“…As there are only three reports of the use of plasma-derived FIX infusions in cardiac surgery, it is difficult to compare the total unitage of FIX used in our case to these single cases of patients with haemophilia of differing weight and baseline FIX levels [24,29,30]. Previous groups using CI have aimed for lower target FIX levels [24,30], often significantly lower than our target of 1.00 IU mL )1 , and consequently had lower FIX requirements. 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].…”

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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“…Reports are widely varied regarding whether recombinant factors should be bolused or given as continuous infusions to achieve the desired level. The use of antiplatelet agents, chemical DVT prophylaxis, and systemic anticoagulation (in the case of valve repair or replacement) is also inconsistent, though all have been reported with good outcomes in select patients 3,4,6,8‐10,12 …”

Section: Discussionmentioning

confidence: 99%

Robotic mitral valve repair in a type B hemophiliac

et al. 2020

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Hemophilia B is a rare X-linked recessive disorder that places surgical patients at an increased risk of bleeding. Patients with hemophilia are now achieving near-normal life expectancies and therefore the number of these patients requiring cardiac surgery due to the development of age-related cardiovascular disease may increase.We present the case of a young male with hemophilia B who was diagnosed with severe symptomatic mitral regurgitation and underwent successful robotic mitral valve repair. To our knowledge, this is the first report of a patient with hemophilia B who underwent robotic mitral valve repair. K E Y W O R D S replacement, valve repair

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Aortic and Mitral Valve Replacement in a Patient with Hemophilia B

Cited by 7 publications

References 6 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

Robotic mitral valve repair in a type B hemophiliac

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