Слайд 2Thrombophilia –
as a trigger of pulmonary embolism
Слайд 4Definitions
Is an inherited or acquired tendency to form blood clots,
commonly in the veins, and, in some cases, in the
arteries. Blood clots in veins usually
Venous thromboembolism (VTE), including
(DVT) and (PE), is
the third most common cardiovascular disease,.
Слайд 5Blood clots in arteries can cause heart attacks, strokes, or
blocked blood flow to an arm or leg. A variety
of thrombophilias,
also referred to as clotting disorders or hypercoagulable states, exist.
Слайд 7Most people who have one of these thrombophilias never
develop a
blood clot.
Thrombophilias can contribute to blood clot formation,
but they are
typically not the only reason for the clot.
Слайд 9Types of thrombophilia
Congenital
Слайд 10Acquired
Heparin-induced thrombocytopenia
Cancer, particularly when metastatic .
Nephrotic syndrome,
Paroxysmal nocturnal hemoglobinuria
, sickle-cell disease
myeloproliferative disorders,
polycythemia vera (excess
red blood cells) and essential thrombocytosis (excess platelets)
antiphospholipid syndrome autoimmune disease.
Слайд 11Antiphospholipid syndrome
Your body produces antibodies that attack phospholipids, fat
molecules thought to keep blood at the right consistency.
The antibodies bind
to the phospholipids, increasing your risk of a blood clots.
Слайд 13Symptoms of thrombophilia
Most people with thrombophilia don't have symptoms and
never have health problems. Symptoms only occur if thrombophilia causes
a blood clot.
If you have thrombophilia, you're at increased risk of developing a DVT or pulmonary embolism.
Слайд 14Warning signs of DVT include:
pain, swelling and tenderness in your leg
(usually in your calf)
a heavy ache in the affected area
warm
skin in the area of the clot
red skin, particularly at the back of your leg below the knee;;
Слайд 15Part of the blood clot can sometimes break away and
travel through the bloodstream.
This can be dangerous because the
clot becomes lodged in the lungs. Known as a pulmonary embolism,
this serious and potentially life-threatening condition can prevent blood reaching your lungs.
Thrombophilia as trigger of pulmonary embolism
Слайд 16chronic thromboembolic pulmonary hypertension (CTEPH) is
form of pre-capillary pulmonary hypertension
which results
after pulmonary obstruction with
thrombus and organised fibrous tissue,
accompanied by pulmonary arteriopathy.
Слайд 17
The symptoms of a PE are:
chest or upper back pain
shortness of breath
coughing –
usually dry, but you may cough up blood or mucus
containing blood
Light headedness or dizziness fainting
symptoms of significant residual perfusion defects .
Chronic thromboembolic pulmonary hypertension
• patient have decreased exercise tolerance, dyspnoea
Слайд 18Diagnosing thrombophilia
If you develop a blood clot, you may be
tested for thrombophilia a few weeks or months later. A
blood sample is taken to look for chemical imbalances.
Слайд 19Thrombophilia testing may be considered in the following scenarios.
1. A
person with venous blood clots in the legs or lungs
(DVT or PE)
a. Clot associated with only a mild trigger (minor surgery, minor immobility or short-distance travel, birth-control pill, patch or ring).
.
2. A person with an unexplained blood clot in a vein in an unusual location (such as in the veins of the abdomen or surrounding
the brain).
Слайд 203. A person who has never had a clot, but
who has a first-degree relative with a strong thrombophilia (mother,
father,
sister, brother, child;
4. Unexplained arterial blood clot in a young person.
5. Recurrent miscarriages with no other cause.
Слайд 21The risk factors for provoked PE include
1 -age, personal history
of VTE,
2- active malignancy,
3-Hormone replacement therapy, oral 4-contraception,
5 -immobilization,
and surgery or trauma within the last 3 months [
If unprovoked PE diagnosed, then thrombophilia testing done
Слайд 22Tests for thrombophilia include
1-complete blood count
2-prothrombin time,
3-partial thromboplastin time,
4-thrombodynamics test,
5-thrombin time and
6-reptilase time,
Слайд 23Common thrombophilia tests.
Activated protein C and factor V Leiden genetic
test
• Prothrombin (factor II) genetic test
• Protein C activity
• Protein
S activity, free protein S antigen
• Antithrombin activity
Слайд 24•Antiphospholipid,, Anticardiolipinn and
Anti-beta-2-glycoprotein I antibodies.
Lupus anticoagulant
• Other tests: In
patients with unexplained clots in the abdomen: JAK-2 mutation,
•
In young patients (<30 years old) with unexplained vein or artery clots: homocysteine
- D DIMER TEST ALSO DONE D-dimer is a fibrin degradation product,.
Слайд 26
Current tests for thrombophilia have limitations. They may be able
to help identify the condition, but they can't always determine
the cause of an increased blood clotting tendency
VQ SCAN [VENTILATION PERFUSION SCAN ;
D-DIMER
;ECHOCARDIOGRAPHY ;
CT PULMONARY ANGIOGRAM ,
chronic thromboembolic pulmonary hypertension (CTEPH)
Слайд 28PULMONARY EMBOLISM DIAGNOSIS FLOWCHART
Слайд 30Many people with thrombophilia won't need treatment unless it causes
thrombotic illness.
Слайд 31There is no specific treatment for thrombophilia, unless it is
caused by an underlying medical illness (such as nephrotic syndrome),
where the treatment of the underlying disease is needed.
Слайд 32This will depend on the type of thrombophilia you have
and factors such as your age, weight, lifestyle and family
history.
You may need to take warfarin tablets or have an injection of heparin.
warfarin It takes a few days to work properly.
Слайд 34If you have a clot and need immediate treatment, you'll
usually be given heparin injections for a few days alongside
warfarin – the heparin injections will work straight away.
Слайд 35A heparin injection may be given on its own to
prevent clots forming, and may also be used to treat
people with thrombophilia or antiphospholipid syndrome before and after surgery or during pregnancy.
Слайд 36Unlike warfarin, heparin is safe to take in pregnancy. Both
warfarin and heparin are safe to use while breastfeeding
Слайд 37International normalised ratio (INR) test
Regular blood test measure your
blood clotting ability while taking warfarin.
The INR test will be
needed less frequently once your ideal dose has been reached – an INR of 2-3 is usually the aim.
Слайд 38However, DIRECT ORAL ANTICOAGULANTS should
not be used during pregnancy or
breastfeeding, in
which case low molecular weight heparin is indicated
Слайд 39Edoxaban and rivaroxaban and dalteparin in cancer-related
thrombosis,
but are associated with
an increased risk for major
Bleeding.
Слайд 40Oral factor Xa inhibitors (eg, apixaban, rivaroxaban)
are preferred to dabigatran
or warfarin to treat proximal
DVT and PE because they do
not require parenteral
anticoagulation for initiation
Слайд 42Treatment for pulmonary embolism -
ranges from medical therapies aiming to
vasodilate the pulmonary vasculature to pulmonary endarterectomy.
Слайд 43Thrombolysis or surgical
embolectomy, catheter-based intervention
IVC [INFERIOR VENA CAVA filter] insertion
may prevent PE in patients with acute VTE and an
absolute
contraindication to anticoagulation,
For patients with massive PE (sustained hypotension)
Слайд 45Anticoagulation does not prevent POST THROMBOTIC SYNDROME.
Thrombolysis and
elastic compression stockings .
Useful only to control symptoms of
leg swelling and pain.
Слайд 47Lifestyle advice
.
following precautions to lower your risk of developing blood
clots:
lose weight if you're overweight
stop smoking
eat a healthy, balanced diet and exercise regularly
avoid being
immobile for long periods – being inactive can cause a DVT