a bleeding diathesis has been recognized in pt. with cchd, a variety of coagulation abnormalities...
TRANSCRIPT
A bleeding diathesis has been recognized in pt. with CCHD, a variety of coagulation abnormalities has been postulated:
1- Polycythemia 2- Hyper viscosity.3- DIC 4- Platelet
function abnormalities.5- Decreased production of coagulation
factors.6- Vitamin K deficiency.7- Primary fibrinolysis.
Pathophysiology
Arterial hypoxia Erythropoietin
RBC Blood viscosity
Decompensated erythrocytosis.( as
increased blood viscosity limiting factor in
tissue oxygenation.)
Polycythemia
#Headache.#Fatigue.#Dizziness.#Visual disturbance.#Paresthesia.#myalgia.#Irritability.
Hyper viscosity (C/P)
Hyper viscosity
Hyper viscosity symptoms occur
usually at the level of packed
cell volume much lower than that
known to produce, it due to the
associated iron deficiency anemia
which is common in cyanotic
infants (dietary).
Polycythemia increased
viscosity
Decrease blood flow & tissue
perfusion (Vascular stasis)
Intravascular deposition of fibrin
and platelet Consumption
of platelet coagulation factors
(DIC) Increase Risk of
bleeding
DIC
Primary fibrinolysis due to coagulation abnormalities usually occur in cyanotic infants.
Fibrinolysis
Laboratory Tests for hemotatic abnormalities PT,
PTT are commonly longer in patients with
haematocrit value >60%, However >50% of
neonates had abnormal coagulation profile
even in the absence of Polycythemia (Due to
impaired synthesis and activation of factor II, vII,
IX, X, because of Vit.K deficiency).
Impaired production of coagulation factor
*Haemodilution resulting from high priming volume.
*Delayed hepatic maturation secondary to poor organ perfusion.
*Complex operative Procedures requiring long duration of CPB.
*Multiple extracardiac lesions.
Other causes of bleeding
in CCHD
-Preoperative Phlebotomy was first suggested
for CCHD Patients in 1964.
-In older children (>5years old) 500 ml of blood
over 30 to 45 min followed by an equivalent
volume of isotonic saline, this may be followed
every 24hrs by an additional 500ml
phlebotomy until HB level of <65% is
achieved.
Phlebotomy
Phlebotomy*Is recommended with symptomatic
hyper- viscosity when dehydration is
not the cause (Hb>65%).
*The red blood cell reduction in CCHD
improved platelet aggregation & lessen
the risk of perioperative bleeding.
Phlebotomy
RBC mass risk of
cerebrovascular events (as a result of
reduced CBF, secondary to hyper
viscosity) therefore Phlebotomy reduce
the risk of cerebral infarction.
If Symptom of hyperviscosity does not improve
after phlebotomy
Possibility of
concomitant iron deficiency anemia.
(as iron deficient red blood cells are less
deformable than normal RBC and does not
pass through the microcirculation).
NB:
A-Pharmacological approach:
Aprotinin:
Is a non specific serine protease inhibitors that
inhibits fibrinolysis and complement
activation due to its effect on the
kallikrein/kinin system.
Strategies to decrease blood loss postoperatively
1-Expensive.
2-Thrombus formation.
3-severe hemodynamic instability.
4-Impaired renal function.
5-Risk of anaphylaxis(due to IgG, IgE antibodies).
Disadvantage:
(EACA) is a synthetic agent that inhibit the
fibrinolytic system by inhibiting activation of
plasminogen (EACA) is used in a dose of
100mg /kg after anesthetic induction,
100mg /kg after in the CPB pump prime and
100mg / on weaning from CPB over 3 hrs.
Aminocaproic acid
*Synthetic antifibrinolytic it act by
effectively inhibiting fibrinolysis.
*Is used in a single dose of 50mg/kg
after skin incision.
Tranexanmic acid
Repeated median sternotomy techniqueit is a major challenge because it can be
associated with ventricular or vascular surgery injury to overcome this problem precaution should be taken as:
1-Avoid sudden separation of sternum.
2-Avoid sharp dissection.
3-Elimination of electrocautary during lysis of adhesions.
4-Avilability of fresh blood.
5-Alternative approach to the femorofemoral bypass before sternotomy
B-Surgical technique modification
Ultrafiltration of the extracorporeal
circuit volume after separation from
CPB with reinfusion of the salvage
concentrate.
C-CPB Modification(ultrafiltration during CPB)
1-Means of blood conservation.
2-Can attenuate the inflammatory response to CPB that lead to tissue edema and multiple organ dysfunction.
3-It lead to decrease interleukin I, interleukin 6, interleukin 8 and myloperoxidase this lead to decrease postoperative blood loss, time to extubation, postoperative alveolar arterial oxygen gradient.
Advantage of ultrafiltration
Despite taking necessary precautions
excessive bleeding can occur after
surgery and treatment needs to be
initiated after proper surgical hemostasis
and adequate heparin neutralization have
been achieved.
Management of excessive bleeding after surgery
Laboratory tests may be required to identify the
haemostatic abnormality to guide proper
therapy while waiting for the laboratory
results, Fresh blood <48hrs should be
transfused in children <2yrs old, in children
>2yrs old platelet concentrate followed by
FFP should be used and give better results
than fresh whole blood.
NB: