Management of severe perioperative bleeding

64 Pages·2017·0.78 MB·English

Eur J Anaesthesiol 2017; 34:332–395
GUIDELINES
Management of severe perioperative bleeding: guidelines
from the European Society of Anaesthesiology
First update 2016
Sibylle A. Kozek-Langenecker, Aamer B. Ahmed, Arash Afshari, Pierre Albaladejo, Cesar Aldecoa,
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on FromtheDepartmentofAnaesthesiology&IntensiveCare,EvangelicalHospitalVienna,Vienna,Austria(SAKL),DepartmentofAnaesthesiology&IntensiveCare,Glenfield
06/07/2018 HARniooaseHpsiottarhtle,esgLiaoe,liocVgeasyllta&edr,IonlUtidenn,isSteipvdeaiKCnian(CrgedA,o)C,mDHeU(ApDaBreAtm)G,erDnetneoopfbaGrletmeHneeoˆnrptailtoaSfl,uAMrgniceahreyas,ltlLhoietnhs,uiGoalnoreiganyno,bUUlenn,ivivFeerrarssnitictyyeoH(fPHoAse)pa,ilDttahelSpoacfrietCmnocepenest,noKhfaaAgunneaanes,s,CtLhoiethpsuieoanlonhgiaayg(e&GnBI,n)Dt,eDennsemipveaarrCtkma(erAenA,tH,ofJoSAsp)n,iatDaelseUtphnaeirsvtemiorelsonitgatyroi&of
IntensiveCare,UniversityHospital‘FedericoII’,Napoli,Italy(EDR),DepartmentofAnaesthesiology,BostonChildren’sHospital,Boston,Massachusetts,UnitedStates
(DFa),DepartmentofAnaesthesiology&IntensiveCare,EmergencyInstituteforCardiovascularDisease,Bucharest,Romania(DCF),DepartmentofAnaesthesiology,
UniversityHospitalofInnsbruck,Innsbruck,Austria(DFr),DepartmentofAnaesthesiology,Children’sUniversityHospitalZurich,Zu¨rich,Switzerland(TH),Departmentof
Anaesthesiology&IntensiveCare,KlinikumStraubing,Straubing,Germany(MJ),DepartmentofAnaesthesiology&PainMedicine,MaastrichtUniversityMedicalCentre,
Maastricht,theNetherlands(MDL),DepartmentofAnaesthesiology&IntensiveCare,HospitalClinicoUniversitarioValencia,Valencia,Spain(JVLP),Departmentof
Anaesthesia,RoyalFreeHospital,London,UnitedKingdom(SM),DepartmentofAnaesthesiology&IntensiveCare,GeneralHospitalLinz,Linz,Austria(JM),Departmentof
Anaesthesiology&IntensiveCare,UniversityHospitalofSzeged,Szeged,Hungary(ZLM),DepartmentofAnaesthesiology&IntensiveCare,FranziskusHospital,Bielefeld,
Germany(NRM),DepartmentofAnaesthesiology&IntensiveCare,GroupeHospitalierCochin,Paris,France(CMS),DepartmentofAnaesthesiology,CHUBrugmann,
Brussels,Belgium(PJFVDL),DepartmentofAnaesthesiology,HerlevUniversityHospital,Herlev,Denmark(AJW),DepartmentofAnaesthesiology,GhentUniversity
Hospital,Ghent,Belgium(PWo,PWy)andDepartmentofAnaesthesiology&IntensiveCare,UniversityFrankfurt/Main,FrankfurtamMain,Germany(KZ)
CorrespondencetoSibylleA.Kozek-Langenecker,DepartmentofAnaesthesiology&IntensiveCare,EvangelicalHospitalVienna,Hans-Sachs-Gasse10-12,1180
Vienna,Austria;E-mail:[email protected]
0265-0215Copyright#2017EuropeanSocietyofAnaesthesiology.Allrightsreserved. DOI:10.1097/EJA.0000000000000630
Copyright © European Society of Anaesthesiology. Unauthorized reproduction of this article is prohibited.
Managementofsevereperioperativebleeding 333
CONTENTS
1 Summary: Recommendations, Suggestions and Statements . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 334
1.1 Evaluation of coagulation status . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 334
1.2 Preoperative and postoperative correction of anaemia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 334
1.3 Optimising circulation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 335
1.4 Oxygen fraction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 335
1.5 Monitoring tissue perfusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 335
1.6 Transfusion of labile blood products . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 335
1.7 General coagulation management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 336
1.8 Algorithms in specific clinical fields . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 337
1.9 Antithrombotic drugs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 338
1.10 Comorbidities involving haemostatic derangement . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 340
1.11 Patients with congenital bleeding disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 340
2 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 341
3 Methods . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 342
3.1 Task force selection . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 342
3.2 Search for evidence . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 342
3.3 Guideline preparation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 342
4 Evaluation of coagulation status . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 343
4.1 Perioperative coagulation testing . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 343
4.2 Evaluation of platelet function . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 345
5 Anaemia management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 346
5.1 Preoperative correction of anaemia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 346
6 Optimising circulation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 348
6.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 348
6.2 Evidence-based medicine and perioperative fluid therapy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 349
6.3 Optimising macrocirculation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 349
6.4 Considerations for microcirculation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 349
7 Transfusion of labile blood products . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 350
7.1 Infectious risk of allogeneic blood components. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 350
7.2 Immunological and non-immunological complications associated with the transfusion of labile
blood components. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 351
7.3 Storage lesions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 353
7.4 Cell salvage . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 353
7.5 Plasma and platelet transfusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 354
8 General coagulation management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 354
8.1 Indications, contraindications, complications and doses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 354
8.2 Correction of confounding factors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 355
8.3 Cost implications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 355
9 Algorithms in specific clinical fields . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 355
9.1 Cardiovascular surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 355
9.2 Gynaecological (non-pregnant) surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 359
9.3 Obstetric bleeding . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 359
9.4 Orthopaedic surgery and neurosurgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 362
9.5 Paediatric surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 363
9.6 Visceral and transplant surgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 363
10 Antithrombotic drugs . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 364
10.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 364
10.2 Antiplatelet agents . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 365
10.3 Anticoagulant agents . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 366
11 Comorbidities involving haemostatic derangement . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 369
11.1 Patients with comorbidities involving haemostatic derangement . . . . . . . . . . . . . . . . . . . . . . . . . . . 369
11.2 Patients with congenital bleeding disorders . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 371
Acknowledgements relating to this article. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 379
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 380
Eur J Anaesthesiol 2017; 34:332–395
Copyright © European Society of Anaesthesiology. Unauthorized reproduction of this article is prohibited.
334 Kozek-Langeneckeretal.
1. Summary: recommendations, suggestions 1.1.1. Evaluation of platelet function
and statements Wesuggestpreoperativeplateletfunctiontestingonlyin
Gradeofrecommendationshowninboldtype(Table1) association with a positive bleeding history. 2B
Wesuggestthatpreoperativeplateletfunctiontestingbe
used to identify decreased platelet function caused by
1.1. Evaluation of coagulation status medical conditions or antiplatelet medication. 2B
Before surgery or invasive procedures, we recommend
Bleedingtimeisinfluencedbymanyvariablesandisnot
theuse ofastructured patient interview orstandardised
useful for stratifying bleeding risk. C
questionnairewhichconsidersclinicalandfamilybleed-
ing history and detailed information on the patient’s
medication. 1C
1.2. Preoperative and postoperative correction
We recommend the use of standardised questionnaires of anaemia
onbleedinganddrughistoryaspreferabletotheroutine Preoperativeanaemiainadultsandchildrenappearstobe
use of conventional coagulation screening tests such as a strong predictor for perioperative blood transfusion
activated partial thromboplastin time (aPTT), inter- across various types of conditions and surgeries and
national normalised ratio (INR) and platelet count in may be associated with adverse events. B
elective surgery. 1C
We recommend that patients at risk of bleeding are
We recommend the application of intervention algor- assessed for anaemia 3 to 8 weeks before surgery. 1C
ithms incorporating pre-defined triggers and targets
Ifanaemiaispresent,werecommendidentifyingthecause
based on viscoelastic haemostatic assay (VHA) coagu-
(irondeficiency,renalinsufficiencyorinflammation).1C
lation monitoring to guide individualised haemostatic
intervention in the case of perioperative bleeding. 1C Werecommendtreatingirondeficiencywithironsupple-
mentation. 1B
IfVHAisnotavailablewerecommendtheapplicationof
intervention algorithms incorporating pre-defined trig- Werecommendtheuseofintravenousironinpreference
gers based on conventional coagulation tests. 1C to oral iron. 1C
Table1 Gradesofrecommendation – GradingofRecommendationsAssessment,DevelopmentandEvaluationsystem
Clarityofrisk/benefit Qualityofsupportingevidence Implications
1AStrongrecommendation. Benefitsclearlyoutweighrisk Consistentevidencefromwellperformed Strongrecommendation,canapplyto
High-qualityevidence. andburdens,orviceversa. randomised,controlledtrialsoroverwhelming mostpatientsinmostcircumstances
evidenceofsomeotherform.Further withoutreservation.
researchisunlikelytochangeourconfidence
intheestimateofbenefitandrisk.
1BStrongrecommendation. Benefitsclearlyoutweighrisk Evidencefromrandomised,controlledtrialswith Strongrecommendation,likelytoapplyto
Moderate-qualityevidence. andburdens,orviceversa. importantlimitations(inconsistentresults, mostpatients.
methodologicalflaws,indirectorimprecise),
orverystrongevidenceofsomeotherform.
Furtherresearch(ifperformed)islikelytohave
animpactonourconfidenceintheestimateof
benefitandriskandmaychangetheestimate.
1CStrongrecommendation. Benefitsappeartooutweigh Evidencefromobservationalstudies, Relativelystrongrecommendation;might
Low-qualityevidence. riskandburdens,orvice unsystematicclinicalexperience,orfrom changewhenhigherqualityevidence
versa. randomised,controlledtrialswithserious becomesavailable.
flaws.Anyestimateofeffectisuncertain.
2AWeakrecommendation. Benefitscloselybalancedwith Consistentevidencefromwellperformed, Weakrecommendation,bestactionmay
High-qualityevidence. risksandburdens. randomised,controlledtrialsoroverwhelming differdependingoncircumstancesor
evidenceofsomeotherform.Further patientsorsocietalvalues.
researchisunlikelytochangeourconfidence
intheestimateofbenefitandrisk.
2BWeakrecommendation. Benefitscloselybalancedwith Evidencefromrandomised,controlledtrialswith Weakrecommendation,alternative
Moderate-qualityevidence. risksandburdens, importantlimitations(inconsistentresults, approacheslikelytobebetterforsome
someuncertaintyinthe methodologicalflaws,indirectorimprecise), patientsundersomecircumstances.
estimatesofbenefits, orverystrongevidenceofsomeotherform.
risksandburdens. Furtherresearch(ifperformed)islikelytohave
animpactonourconfidenceintheestimateof
benefitandriskandmaychangetheestimate.
2CWeakrecommendation. Uncertaintyintheestimatesof Evidencefromobservationalstudies, Veryweakrecommendation;other
Low-qualityevidence. benefits,risksand unsystematicclinicalexperience,orfrom alternativesmaybeequallyreasonable.
burdens;benefitsmaybe randomised,controlledtrialswithserious
closelybalancedwith flaws.Anyestimateofeffectisuncertain.
risksandburdens.
Eur J Anaesthesiol 2017; 34:332–395
Copyright © European Society of Anaesthesiology. Unauthorized reproduction of this article is prohibited.
Managementofsevereperioperativebleeding 335
Ifothercausesofanaemiahavebeenexcludedortreated, 1.4. Oxygen fraction
we suggest erythropoietin-stimulating agents. 2B We recommend that the inspiratory oxygen fraction
should be high enough to prevent arterial hypoxaemia
If autologous blood donation is performed, we suggest
inbleedingpatients,whileavoidingexcessivehyperoxia
treatment with iron and/or erythropoietin-stimulating
[PaO >26.7 kPa (200 mmHg)]. 1C
agentstoavoidpreoperativeanaemiaandincreasedover- 2
all transfusion rates. 2C
1.5. Monitoring tissue perfusion
In patients with preoperative anaemia, we recommend We recommend repeated measurements of a combi-
the use of combined therapy with intravenous iron and nationofhaematocrit (Hct)/haemoglobin, serumlactate,
erythropoietinalongwitharestrictivetransfusionpolicy. and base deficit to monitor tissue perfusion, tissue
1C oxygenationandthedynamicsofbloodlossduringacute
bleeding. These parameters can be extended by
Innon-cancerpatientswithpreoperativeanaemiasched-
measurement of cardiac output, dynamic parameters of
uledforelectivemajorsurgery,werecommendpostpon-
volumestatus[e.g.strokevolumevariation(SVV),pulse
ing surgery until anaemia has been corrected. 1C
pressure variation (PPV)], CO gap and central venous
2
In patients who are anaemic following surgery, we oxygen saturation. 1C
suggest the use of intravenous iron. 2C
1.5.1. Normovolaemic haemodilution
1.3. Optimising circulation Wesuggest theuseofacute normovolaemic haemodilu-
We recommend aggressive and timely stabilisation of tion (ANH) in selected settings. 2C
cardiac pre-load throughout the surgical procedure, as
We recommend against ANH in combination with con-
this appears beneficial to the patient. 1B
trolled hypotension. 1B
In cases of uncontrolled bleeding we suggest lower
In patients with pre-existing or acquired coagulopathy
thresholds for cardiac pre-load and/or permissive hypo-
we suggest that the use of ANH is considered
tension may be considered. 2C
carefully. 2C
Werecommendtheavoidanceofhypervolaemiasecond-
ary to crystalloids or colloids to a level exceeding the
1.6. Transfusion of labile blood products
interstitial space in steady state, and beyond an optimal
We recommend that all countries implement national
cardiac pre-load. 1B
haemovigilance quality systems. 1B
We recommend against the use of central venous pres-
Werecommendarestrictivetransfusionstrategywhichis
sure (CVP) and pulmonary artery occlusion pressure as
beneficial in reducing exposure to allogeneic blood pro-
theonlyvariablestoguidefluidtherapyandoptimisation
ducts. 1A
ofpre-loadduringseverebleeding.Dynamicassessment
offluidresponsivenessandnon-invasivemeasurementof We recommend pathogen inactivation for fresh frozen
cardiac output should be considered instead. 1B plasma (FFP) and platelets. 1C
We suggest the replacement of extracellular fluid losses We recommend that labile blood components used for
with isotonic crystalloids in a timely and protocol-based transfusion are leukodepleted. 1B
manner. 2C
We recommend that blood services implementstandard
Comparedwithcrystalloids,haemodynamicstabilisation operating procedures for patient identification and that
with iso-oncotic colloids, such as human albumin and staff be trained in early recognition of, and prompt
hydroxyethyl starch, causes less tissue oedema. C response to, transfusion reactions. 1C
Infusionofcolloidsinpatients with severebleedingcan We recommend a male-only donor policy for plasma-
aggravatedilutionalcoagulopathybyadditionaleffectson containing blood products to prevent the onset of
fibrin polymerisation and platelet aggregation. C transfusion-related acute lung injury (TRALI). 1C
Wesuggesttheuseofbalancedsolutionsforcrystalloids We recommend that all red blood cell (RBC), platelet
and as a basic solute for iso-oncotic preparations. 2C and leukocyte donations from first-degree or second-
degree relatives be irradiated even if the recipient is
1.3.1. Transfusion triggers immunocompetent,andallRBC,plateletandleukocyte
Werecommendatargethaemoglobinconcentrationof7 products be irradiated before transfusing to at-risk
to 9g dl(cid:2)1 during active bleeding. 1C patients. 1C
Continuous haemoglobin monitoring can be used as a Allogeneic blood transfusion is associated with an
trend monitor. C increased incidence of nosocomial infections. B
Eur J Anaesthesiol 2017; 34:332–395
Copyright © European Society of Anaesthesiology. Unauthorized reproduction of this article is prohibited.
336 Kozek-Langeneckeretal.
1.6.1. Storage lesions We recommend treatment of hypofibrinogenaemia in
WerecommendthatRBCsshouldbetransfusedaccord- bleeding patients. 1C
ingtothefirst-in,first-outmethodinthebloodservicesto
Wesuggestaninitialfibrinogenconcentratedoseof25to
minimise wastage of erythrocytes. 1A
50mgkg(cid:2)1. 2C
1.6.2. Cell salvage In cases wherein fibrinogen concentrate is not available
We recommend the use of red cell salvage which is we suggest cryoprecipitate at aninitialdoseof4 to6ml
helpful for blood conservation in major cardiac and kg(cid:2)1. 2C
orthopaedic surgery. 1B
Plasmatransfusionaloneisnotsufficienttocorrecthypo-
Werecommendagainsttheroutineuseofintraoperative fibrinogenaemia. C
platelet-rich plasmapheresis for blood conservation
In cases of bleeding and low factor XIII activity (e.g.
during cardiac operations using cardiopulmonary bypass
<30%) we suggest administrationof factor XIII concen-
(CPB). 1B trate (30 IU kg(cid:2)1). 2C
Werecommendthatcellsalvageisnotcontraindicatedin
In severe perioperative bleeding we recommend that
bowel surgery, provided that the initial evacuation of
patientsonvitaminKantagonists(VKAs)shouldbegiven
soiled abdominal contents is undertaken, additional cell
prothrombin complex concentrate (PCC) and intrave-
washingisperformedandbroad-spectrumantibioticsare
nous vitamin K before any other coagulation manage-
used. 1C
ment steps. 1B
We suggest that cell salvage is not contraindicated in
Prolonged INR/prothrombin time (PT) or VHA clotting
cancersurgery,providedthatbloodaspirationclosetothe
times alone are not an indication for PCC in bleeding
tumour site is avoided and leukodepletion filters are
patients not on oral anticoagulant therapy. C
used. 2C
Werecommendagainsttheprophylacticuseofrecombi-
1.6.3. Plasma and platelet transfusion nantactivatedfactorVII(rFVIIa)duetoincreasedriskof
Werecommendagainsttheuseofplasmatransfusionfor fatal thrombosis. 1B
pre-proceduralcorrectionofmild-to-moderatelyelevated
Wesuggestthatoff-labeladministrationofrFVIIacanbe
INR. 1C
consideredforlife-threateningbleedingwhichcannotbe
We recommend early and targeted treatment of coagu- stoppedbyconventional,surgicalorinterventionalradio-
lationfactordeficienciesintheplasma.Sourcesofcoagu- logical means and/or when comprehensive coagulation
lation factors are coagulation factor concentrates, therapy fails. 2C
cryoprecipitate or high volumes of plasma, depending
We recommend tranexamic acid to prevent bleeding
on the clinical situation, type of bleeding, type of
during major surgery and/or treat bleeding due to (or
deficiency and resources provided. 1B
at least suspected) hyperfibrinolysis (e.g. a dose of 20 to
In the treatment of acquired coagulation factor 25mgkg(cid:2)1). 1B
deficiency,wesuggesttheconsiderationofaratio-driven
We suggest the use of desmopressin (DDAVP) under
protocol (RBC:plasma:platelet concentrates) early in
specific conditions [acquired von Willebrand syndrome
uncontrolled massive bleeding outside the trauma
(VWS)]. 2C
setting followed by a goal-directed approach as soon as
possible. 2C Based on the current literature there is no evidence to
recommend antithrombin supplementation in elective
We suggest coagulation factor concentrates for the
surgical patients while they are bleeding.
primary treatment of acquired coagulation factor
deficiency due to their high efficacy and their minimal We recommend structured staff education and training.
infectiousness. 2C 1C
We recommend against indiscriminate use of plasma
transfusion in perioperative bleeding management. 1C
1.7.1. Correction of confounding factors
We suggest platelet concentrate transfusion in bleeding
Werecommendmaintainingperioperativenormothermia
situations clearly related to antiplatelet drugs or throm-
because it reduces blood loss and transfusion require-
bocytopaenia less than 50(cid:3)109 l(cid:2)1. 2C
ments. 1B
1.7. General coagulation management We recommend that pH correction should be pursued
Fibrinogen concentration of less than 1.5 to 2g l(cid:2)1 is during treatment of acidotic coagulopathy, although pH
considered as hypofibrinogenaemia in acquired coagulo- correction alone cannot immediately correct acidosis-
pathy and is associated with increased bleeding risk. C induced coagulopathy. 1C
Eur J Anaesthesiol 2017; 34:332–395
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Managementofsevereperioperativebleeding 337
We recommend that rFVIIa should only be considered In complex cardiovascular surgery we recommend fibri-
alongside pH correction. 1C nogenconcentrateinfusionguidedbyVHAmonitoringto
reduce perioperative blood loss. 1B
We recommend that calcium should be administered
during massive transfusion if calcium concentration is We suggest that rFVIIa may be considered for patients
low, to preserve normocalcaemia (>0.9mmol l(cid:2)1). 1B withintractablebleedingduringandaftercardiovascular
surgeryonceconventionalhaemostaticoptionshavebeen
We suggest that endovascular embolisation is a well
exhausted. 2B
tolerated alternative to open surgical intervention after
failed endoscopic treatment for non-variceal upper We suggest that antiplatelet therapy with aspirin or
gastrointestinal bleeding (UGIB). 2C clopidogrel may be administered in the early postopera-
tive period without increasing the risk of postoperative
We suggest super-selective embolisation as primary
bleeding. 2C
therapyfortreatmentofangiogrampositivelowergastro-
intestinal tract bleeding. 2C We recommend the use of standardised VHA-guided
haemostatic algorithms with pre-defined intervention
We suggest embolisation as first-line therapy for arterial
triggers. 1B
complications in pancreatitis. 2C
1.8.2. Gynaecological (non-pregnant) surgery
1.7.2. Cost implications We suggest that normovolaemic haemodilution should
Both bleeding and transfusion of allogeneic blood pro- notbeused asitdoes notreduce allogeneictransfusion.
ducts independently increase morbidity, mortality, 2B
length of stay in ICU and hospital and costs. B
Cellsalvagemayreduceallogeneictransfusioningynae-
Tranexamicacidcanreduceperioperativebloodlossand cological (including oncological) surgery. B
transfusion requirements; this can be highly cost-effec-
Wesuggestusingpreoperativeintravenousirontoreduce
tive in several major surgical and trauma settings. B
allogeneictransfusionrequirementsinanaemicgynaeco-
We recommend restricting the use of rFVIIa to its logical cancer patients receiving chemotherapy. 2B
licensed indication as, outside these indications, the
Wesuggestusingintravenousirontocorrectpreoperative
effectiveness of rFVIIa to reduce transfusion require-
anaemia in women with menorrhagia. 2B
ments and mortality remains unproven and the risk of
arterialthromboembolicevents,aswellascosts,arehigh. Tranexamic acid may reduce perioperative bleeding in
1A gynaecological cancer surgery. C
Cellsalvagecanbecost-effectiveinselectedpatients.A
1.8.3. Obstetric bleeding
The cost-effectiveness of a ratio-driven transfusion pro- We recommend that peripartum haemorrhage (PPH)
tocol has not been investigated. should be managed by a multidisciplinary team. 1C
Goal-directed therapy with coagulation factor concen- WerecommendedtheuseofanescalatingPPHmanage-
trates (fibrinogen and/or PCC) may reduce transfusion- mentprotocolincludinguterotonicdrugs,surgicaland/or
associated costs in trauma, cardiac surgery and liver endovascular interventions and procoagulant drugs. 1B
transplantation. C
Risk awareness and early recognition of severe PPH are
essential. C
1.8. Algorithms in specific clinical fields
Wesuggestthatpatientswithknownplacentaaccretabe
1.8.1. Cardiovascular surgery
treated by multidisciplinary care teams. 2C
Withdrawal of aspirin therapy increases the risk of cor-
onary thrombosis; continuation of aspirin therapy Cell salvage is well tolerated in obstetric settings, pro-
increases the risk of bleeding. B videdthatprecautionsaretakenagainstrhesusisoimmu-
nisation. C
Withdrawal of clopidogrel therapy increases the risk of
coronarythrombosis;continuationofclopidogreltherapy We suggest that using perioperative cell salvage during
increases the risk of bleeding. A caesarean section may decrease postoperative homolo-
gous transfusion and reduce hospital stay. 2B
We recommend prophylactic administration of tranexa-
mic acid before CPB in patients undergoing coronary Intravenousironsupplementationimprovesfatigueat4,
artery bypass grafting (CABG) surgery. 1A 8 and 12 weeks postpartum. B
We suggest tranexamic acid can be applied topically to Wesuggestassessingfibrinogenlevelsinparturientswith
the chest cavity to reduce postoperative blood loss fol- bleeding,aslevelslessthan2gl(cid:2)1mayidentifythoseat
lowing cardiac surgery. 2C risk of severe PPH. 2B
Eur J Anaesthesiol 2017; 34:332–395
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338 Kozek-Langeneckeretal.
Dynamic platelet count decrease or a level less than We recommend a low CVP and restrictive fluid admin-
100(cid:3)109 l(cid:2)1 at the onset of labour, particularly if com- istration during liver surgery to reduce bleeding. 1B
binedwithplasmafibrinogenlevellessthan2.9gl(cid:2)1,may
We recommend tranexamic acid for treatment of
indicate an increased risk of PPH. C
fibrinolysis (evident from microvascular oozing or
At the beginning of labour aPTT and PT are of little VHA clot lysis measurement) but not for routine
predictive value for PPH. C prophylaxis. Marginal grafts (e.g. donation after
cardiac death) increase the risk of fibrinolysis postre-
VHA can identify obstetric coagulopathy. B
perfusion. 1C
We recommend against pre-emptive fibrinogen replace-
Wesuggestthattranexamicacidshouldbeconsideredin
ment;however,inongoingPPHwithhypofibrinogenae-
cirrhotic patients undergoing liver resection. 2C
mia we recommend fibrinogen replacement. 1C
In severe PPH we suggest a VHA-guided intervention 1.8.7. Acute upper gastrointestinal bleeding
protocol. 2C We recommend that acute variceal bleeding should be
managed by a multidisciplinary team. A specific multi-
We suggest that tranexamic acid be considered before
modal protocol for upper gastrointestinal haemorrhage
caesareansectionandincasesofantepartumbleeding.2B
should be available. 1C
Werecommendtheadministrationoftranexamicacidin
Transjugular intrahepatic portosystemic stent-shunt
PPH at a dose of 1g intravenously (IV) as soon as
(TIPSS) can be suggested as an option for rescue
possible,whichcanberepeatedifbleedingcontinues.1B
therapy after initial medical and endoscopic therapy
fail. 2B
1.8.4. Orthopaedic surgery and neurosurgery
We recommend early interventional endoscopy and the
Reducedplateletactivityisassociatedwithearlyhaema-
immediateuseofvasopressors(somatostatinorterlipres-
toma growth, more intraventricular haemorrhage and
sin) to reduce bleeding. 1B
worse 3-month outcomes following intracranial haemor-
rhage (ICH). C Tranexamicacidreducesmortalitybutnotre-bleeding.B
Lowplatelet count, low plasmafibrinogen concentration
1.8.8. Coagulopathy and renal disease
and factor XIII deficiency are predictive of bleeding
Point-of-care tests of platelet function and bleeding
complicationsinICH,intracranialsurgeryandmajorspine
time provide no reliable platelet function assessment
surgery,particularlywhentheyoccurincombination.C
in uraemia and no prediction of bleeding in this
setting. C
1.8.5. Paediatric surgery
We suggest low-volume sampling for standard coagu- Wesuggestthatconjugatedoestrogentherapyshouldbe
lation tests and VHA-guided interventions. 2C used in uraemia. 2C
We recommend the use of isotonic and balanced resus- WesuggestthatDDAVPshouldbeconsideredforredu-
citation fluids in bleeding children. 1C cing bleeding during surgery and for managing acute
bleeding in uraemic patients. 2C
Exceptforprematurebabiesandcyanoticnewborns,haemo-
globintargetsinbleedingchildrenare7to9gdl(cid:2)1.C 1.9. Antithrombotic drugs
1.9.1. Antiplatelet agents
1.8.6. Visceral and transplant surgery We recommend that aspirin therapy should continue
Despite PT, aPTT and INR indicating coagulopathy in perioperatively in most surgical settings, especially car-
chronic liver disease (CLD), global coagulation tests diac surgery. 1C
(thrombingenerationandVHA)suggestthathaemostasis
Where aspirin withdrawal before surgery is considered,
is balanced in stable CLD. C
we recommend a time interval of 3 days. 1C
Mild-to-moderate prolongation of the preoperative PT
In patients with risk factors for vascular complications
andINRdonotpredictbleedinginpatientswithCLD.C
naı¨ve of any antiplatelet treatment, it is not recom-
We recommend that, in acute liver failure, moderately mended that treatment with aspirin be initiated preo-
elevated INR should not be corrected before invasive peratively. 1B
procedures, with the exception of intracranial pressure
In patients treated chronically with aspirin for the sec-
monitor insertion. 1C
ondarypreventionofcardiovascularevents,exceptthose
Fluid restriction, phlebotomy, vasopressors and transfu- patients with coronary stents, we recommend aspirin
sion protocols may be associated with low transfusion interruption for procedures where there is a very high
rates during orthotopic liver transplant (OLT). C bleeding risk. 1B
Eur J Anaesthesiol 2017; 34:332–395
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Managementofsevereperioperativebleeding 339
Inpatientschronicallytreatedwithaspirinforsecondary 1.9.2. Heparin
prevention of cardiovascular events, we recommend We recommend that severe bleeding associated with
aspirin be maintained during and after low and medium intravenous unfractionated heparin (UFH) should be
bleeding risk procedures. 1B treated with intravenous protamine at a dose of 1mg
per 100 IU UFH given in the preceding 2 to 3h. 1A
Wesuggestcarefulconsiderationofpostoperativebleed-
ing complications when timing the first postoperative We suggest that severe bleeding associated with subcu-
administration and dose of anticoagulants along with taneous (SC) UFH unresponsive to intravenous prota-
resumption of aspirin. 2C mineatadoseof1mgper100IUUFHcouldbetreated
by continuous administration of intravenous protamine,
For intraoperative or postoperative bleeding clearly
with the dose guided by aPTT. 2C
related to aspirin, we suggest that platelet transfusion
beconsidered(dose:0.7(cid:3)1011per10kgbodyweightin We suggest that severe bleeding related to SC low
adults). 2C molecular weight heparin (LMWH) should be treated
with intravenous protamine at a dose of 1mg per 100
We recommend that aspirin be continued for at least 4
antifactorXaunitsofLMWHadministeredand,ifunre-
weeksafterbaremetalstent(BMS)implantationand3to
sponsive, with a further 0.5mg protamine per 100 anti-
12 months after drug-eluting stent (DES) implantation,
factor Xa units. 2C
unless the risk of life-threatening surgical bleeding on
aspirin is unacceptably high. 1A
1.9.3. Fondaparinux
WesuggestthatP2Y12inhibitortreatmentbeconsidered We suggest that the administration of rFVIIa could be
for at least 4 weeks after BMS implantation and 3 to 12 considered to treat severe bleeding associated with SC
months after DES implantation, unless the risk of life- administration of fondaparinux (off-label treatment). 2C
threatening surgical bleeding on this agent is unaccep-
tably high. 2A 1.9.4. Vitamin K antagonists
We recommend that VKAs should not be interrupted in
If clinically feasible, we suggest postponing (semi-
patients undergoing low bleeding risk procedures: skin
urgent) surgery for at least 5 days after cessation of
surgery, dental and oral procedures, gastric and colonic
ticagrelor and clopidogrel, and for 7 days in the case of
endoscopies (even if biopsy is scheduled, but not poly-
prasugrel,unlessthepatientisathighriskofanischaemic
pectomies), nor for most ophthalmologic surgery [i.e.
event. 2B
mainly anterior chamber (cataract)]. 1C
We recommend that antiplatelet agent (APA) therapy
Werecommendthatforlowormoderatethromboticrisk
should resume as soon as possible postoperatively to
patients [e.g. atrial fibrillation patients with CHADS
prevent platelet activation. 1C 2
score (cid:4)4; patients treated for >3 months for a non-
Wesuggestthatthefirstpostoperativedoseofclopidogrel recurrent venous thromboembolism (VTE)] undergoing
orprasugrelshouldbegivennolaterthan24hafterskin procedures requiring INR less than 1.5, VKA should be
closure.Wealsosuggestthatthisfirstdoseshouldnotbea stopped 3 to 5 days before surgery (acenocoumarol,
loading dose. 2C warfarin). No bridging therapy is needed. Measure
INRonthedaybeforesurgeryandgive5mgoralvitamin
We recommend that a multidisciplinary team meeting
K if INR exceeds 1.5. 1C
shoulddecideontheperioperativeuseofAPAsinurgent
and semi-urgent surgery. 1C Werecommendbridgingtherapyforhighthromboticrisk
patients (e.g. atrial fibrillation patients with a CHADS
Wesuggestthaturgentorsemi-urgentsurgeryshouldbe 2
score >4; patients with recurrent VTE treated for less
performed under aspirin/clopidogrel or aspirin/prasugrel
than 3 months; patients with a prosthetic cardiac valve).
combinationtherapyifpossible,oratleastunderaspirin
Warfarin: last dose 5 days before surgery; 4 days before
alone. 2C
surgery, no heparin; 3, 2 and 1 day before surgery,
Wesuggestthatplatelettransfusionbeconsidered(dose: LMWH(lastdose24hbeforesurgery)orSCUFHtwice
0.7(cid:3)1011 per 10kg body weight in adults) in cases of or thrice daily; day 0, surgery. Acenocoumarol: 3 days
intraoperativeorpostoperativebleedingclearlyrelatedto beforesurgery,lastdose;2and1daybeforesurgery,same
clopidogrel or prasugrel. 2C protocol as for warfarin. 1C
Accordingtopharmacologicalcharacteristics,wesuggest WesuggestthatthetherapeuticdoseofLMWHorUFH
thatthemanagementofticagrelormaybecomparableto should be tailored for each patient, depending on the
clopidogrel (i.e. withdrawal interval of 5 days). 2C respective thrombotic and bleeding risks. 2C
Platelet transfusions may be ineffective for treating Werecommendthatforlowbleedingriskpatients,VKAs
bleeding related to ticagrelor if given within 12h of should be restarted during the evening or the day after
the drug’s administration. C the procedure (at least 6h after). Therapeutic doses of
Eur J Anaesthesiol 2017; 34:332–395
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340 Kozek-Langeneckeretal.
LMWHshouldbegivenpostoperativelyuntilthetarget We suggest that for low bleeding risk procedures, when
INR is observed in two following measurements. 1C haemostasis is achieved, DOACs should be recom-
mencedduring theevening after theprocedure(at least
Werecommendthatformoderatetohighthromboticrisk
6h after). 2C
patients,prophylacticdosesofheparin(UFHorLMWH)
shouldbestartedduringtheeveningorthedayafterthe We suggest that for intermediate and high bleeding risk
procedure(atleast6hafter)andgivenforupto48to72h, procedures, prophylactic doses of LMWH or DOACs
andthentherapeuticanticoagulationshouldberesumed. (according to specific indications) should be given post-
VKA can restart at that time or later, only when surgical operativelywhenever VTEprophylaxis is requested and
haemostasis is achieved. 1C then the full therapeutic dose of DOAC should be
resumedupto72hpostoperatively,whensurgicalhaemo-
In VKA-treated patients undergoing an emergency pro-
stasisisachieved.2C
cedure, we recommend that INR must be measured on
the patient’s admission to the hospital, with the admin-
1.10. Comorbidities involving haemostatic
istrationoffour-factorPCCtoreverseVKAanticoagulant
derangement
effects(e.g.ataninitialdoseof25IUfactorIXkg(cid:2)1atan
1.10.1. Systemic, metabolic and endocrine diseases
INR of 4) rather than the transfusion of plasma. 1B
Wesuggestthatpatientswithhaemostaticderangements
InbleedingpatientswhereVKA-inducedcoagulopathyis associated with systemic, metabolic and endocrine dis-
considered a contributing factor, we recommend the easesshouldbemanagedperioperativelyincollaboration
administration of four-factor PCC 25 to 50 IU factor IX with a haematologist. 2C
kg(cid:2)1 plus 5 to 10mg IV vitamin K. 1B
We suggest individualised preoperative discontinuation
IfPCCis notavailable,theninbleedingpatientswhere of selective serotonin reuptake inhibitor (SSRI) treat-
VKA-induced coagulopathy is considered a contributing ment. 2B
factor, we recommend the transfusion of plasma (15 to
We suggest individualised preoperative discontinuation
20mlkg(cid:2)1 plus 5 to 10mg IV vitamin K). 1C
ofantiepilepticagents,suchasvalproicacid,which may
increase bleeding. 2C
1.9.5. Direct oral anticoagulants We do not recommend preoperative discontinuation of
We recommend assessment of creatinine clearance in gingko biloba extracts. 1B
patients receiving direct oral anticoagulants (DOACs)
who are scheduled for surgery. 1B 1.11. Patients with congenital bleeding
disorders
WesuggestthatDOACsshouldonlybewithheldtheday
1.11.1. Preoperative assessment
beforesurgeryforpatientsundergoinglowbleedingrisk
We suggest referring the patient to a haematologist for
procedures such as skin surgery, dental and oral pro-
assessment and planning of the intervention if inherited
cedures, gastric and colonic endoscopies (even if biopsy
bleedingdisorders(IBDs)aresuspectedpreoperatively.2C
is scheduled, but no polypectomies) and most ophthal-
mological surgery. 2C We recommend the use of bleeding assessment tools
(BATs)fordetectingandpredictingtheperioperativerisk
For intermediate and high bleeding risk procedures
ofbleedingbeforesurgeryandinvasiveprocedures.1C
(1) we recommend that rivaroxaban, apixaban and
1.11.2. General perioperative management
edoxaban should not be given for 2 days before
Surgery can be safely performed in patients with IBDs
the procedure (i.e. last oral intake 3 days before),
whenthereisappropriatecarefulpreoperativeplanning,
pending a creatinine clearance (Cockcroft–Gault
appropriatereplacement/substitutiontherapy,andmulti-
formula) above 30 ml min(cid:2)1. No bridging therapy is
disciplinary team management. C
needed. 1C
(2) we recommend that dabigatran should not be given We recommend that patients with IBDs be managed
for3daysbeforetheprocedure(i.e.lastoralintake4 perioperatively in collaboration with a haematologist,
days before), if the creatinine clearance is above 50 preferably in dedicated centres with expertise in coagu-
ml min(cid:2)1 and 4 days before the procedure (i.e. last lation disorders. 1C
oralintake5daysbefore),ifthecreatinineclearance
We suggest preoperative haemostatic correction in
isbetween30and50mlmin(cid:2)1.Nobridgingtherapy
patientswithIBDsdependingonthetypeofsurgery.2C
is needed. 1C
1.11.3. Von Willebrand disease
Wesuggestthatinseverebleedingpatientstreatedwith We recommend DDAVP as a first-line treatment for
dabigatran, a specific antidote (idarucizumab) should be minor bleeding/surgery in patients with von Willebrand
considered. 2C disease (VWD), after a trial testing. The standard
Eur J Anaesthesiol 2017; 34:332–395
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Managementofsevereperioperativebleeding 341
regimen is 0.3mgkg(cid:2)1 dissolved in 50 ml saline and If rFVIIa is given to control perioperative bleeding in
infused IV over 20 to 30 min, repeated every 12 to inherited factor VII deficiency, we suggest lower doses
24h usually for no more than 3 days. 1C (e.g.20to25mgkg(cid:2)1every4to6h)thaninhaemophilia
patients with inhibitors. 2C
We recommend replacement of von Willebrand factor
(VWF)withplasma-derivedproductsformajorbleeding/ There are insufficient data to recommend rFVIIa in
surgery. Treatment regimens are specifiedby published perioperative bleeding for patients with other RBDs.
guidelines. 1C
There are insufficient data to recommend peri-
Wesuggestthatantifibrinolyticdrugsbeusedashaemo- procedural DDAVP or antifibrinolytic drugs in patients
static adjuncts. Treatment regimens are specified by with mild RBDs.
published guidelines. 2C
2. Introduction
1.11.4. Platelet defects
Perioperative bleeding management is a complex and
We suggest that DDAVP be used to prevent/control
changingfieldrequiringmultipleassessmentsandappro-
perioperative bleeding in patients with mild inherited
priate strategies to optimise patient care. There is an
platelet defects. 2C
ongoing drive to find new alternatives to transfusion, a
Wesuggestthatantifibrinolyticdrugsbeusedashaemo- desiretoreduceunnecessaryuseofbloodproductsanda
static adjuncts in procedures involving patients with focustowardsmoreevidence-basedperioperativepractice.
inherited platelet defects. 2C Inthisdynamicareaofmedicineitisimperativetoprovide
healthcare professionals with clinically useful and up-to-
We recommend that rFVIIa treatment should be con-
date data concerning the diagnosis and treatment of
sidered in patients with Glanzmann thrombasthenia
patients with perioperative bleeding. As such, the Euro-
undergoing surgery. 1C
peanSocietyofAnaesthesiology(ESA)stronglysupports
We recommend against routine platelet transfusion in thedevelopmentofhigh-quality,evidence-basedclinical
patients with inherited platelet disorders. 1C practice guidelines to help standardise the approach to
patientcareandtoimproveoverallclinicalpractice.1
1.11.5. Haemophilia A and B
In2013,theESAdevelopedanextensivesetofevidence-
We recommend adequate perioperative replacement based guidelines2 for the management of severe perio-
therapy to ensure well tolerated surgery in haemophilia
perativebleedingwiththeoverallaimofprovidinganup-
patients.1C
to-datereviewandsynthesisoftheevidenceandrecom-
Wesuggest that perioperative replacementtherapy(tar- mendationstohelpguideclinicianstowardssaferandmore
get factor level and duration) in haemophilia patients cost-effective strategiesforminimising severe periopera-
follows published guidelines. 2C tive bleeding and thus maximising blood conservation.
Thecurrent guidelines update providesadditional infor-
We recommend either recombinant products or plasma-
mation to assist the clinician to PREPARE, PLAN and
derived concentrates for perioperative replacement
takeACTION.PREPAREforanypotentialbleedingrisks
therapy in haemophilia patients. 1C
by performing preoperative assessments, particularly to
We suggest that coagulation factors be given periopera- detectanaemiaandallowtimeforitscorrection.PLANfor
tively by continuous infusion. 2C any intraoperative bleeding that may occur by utilising
transfusionalgorithmsthatincorporatepre-definedtrans-
We suggest either rFVIIa or activated PCCs for haemo-
fusiontriggerstohelpguidehaemostaticintervention,by
philia patients with inhibitors. 2C
beingawareofthelimitationsofstandardcoagulationtests
Wesuggestantifibrinolyticdrugsasperioperativeadjunct andbymodifyingtheapproachaccordinglytousepoint-of-
therapy in haemophilia patients. 2C care testing and others. If potential bleeding risks are
known in advance and a plan of treatment is in place,
WesuggestDDAVPasfirst-lineperioperativetherapyin
thenecessaryACTIONcanbesetinmotionasrequired.
patients with mild haemophilia A as long as factor VIII
Becauseoftheincreasingevidenceinthisfield,anupdate
can be raised to an appropriate therapeutic level. 2C
oftheguidelineswasplannedevery2years.
Thisdocumentnotonlydetailstheretainedrecommen-
1.11.6. Rare bleeding disorders
dations, suggestions and statements from the original
There are insufficient data to recommend routine peri-
guidelines published in 20132 but also includes new
operativesupplementationofdeficientfactorsinpatients
recommendations as well as revisions to the wording
with rare bleeding disorders (RBDs).
and grades of some of the original recommendations.
WesuggestthatrFVIIabeusedinperioperativebleeding Additional clinical questions have been included in
due to inherited factor VII deficiency. 2C the update.
Eur J Anaesthesiol 2017; 34:332–395
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