Pulmonary Vascular Disease (TBL)
● Venous thromboembolism is a common cause of death in patients cancer, stroke, and
pregnancy.
● The risk factors include; major surgery, pregnancy/ puerperium, COPD, CCF, fracture,
varicose veins, stroke/spinal cord injury, malignancies (abdominal, pelvic), chemotherapy,
increasing age, previous VTN, thrombotic disorders, trauma, and immobility.
● Pulmonary embolism is of two types:
1. Acute; massive and small/ medium.
2. Chronic.
● Acute:
a. Massive:
● Pathophysiology: major hemodynamic effects, decrease in cardiac output, and
acute RHF.
● Symptoms: Faintness, crushing central chest pain, and severe dyspnea.
● Signs: major circulatory collapse, tachycardia, hypotension, increased JVP,
right ventricular gallop, loud P2, severe cyanosis, and decreased urinary
output.
● CXR: usually normal, may be subtle oligemia.
● ECG: anterior T wave inversion and RBBB.
● ABG: decreased PaO2 and decreased PaCO2, with metabolic acidosis.
● DDx: MI, pericardial tamponade, and aortic dissection.
b. Small/ Medium:
● Pathophysiology: occlusion of segmental pulmonary arteries, and infarction
with or w/o effusion.
● Symptoms: pleuritic chest pain, restricted breathing, and hemoptysis.
● Signs: tachycardia, pleural rub, raised hemidiaphragm, crackles, bloodstained
effusion, and lowgrade fever.
● CXR: pleuropulmonary opacities, pleural effusion, linear shadows, and raised
hemidiaphragm.
● ECG: sinus tachycardia.
● ABG: may be normal, or low PaO2 with normal or low PaCO2.
● DDx: pneumonia, pneumothorax, musculoskeletal chest pain.
● Chronic:
■ Pathophysiology: chronic occlusion of pulmonary microvasculature, and RHF.
■ Symptoms: exertional dyspnea, late symptoms of pulmonary HTN or RHF.
■ Signs: minimal in early disease, later there is right ventricular heave, terminal,
there are signs of RHF.
■ CXR: enlarged pulmonary trunk, enlarged heart, prominent right ventricle.
■ ECG: right ventricular hypertrophy and strain.
■ ABG: exertional decrease of PaO2 or desaturation on formal exercise testing.
■ DDx: other causes of pulmonary HTN.
● Investigations:
a. CXR: used to exclude other causes of chest pain; pneumonia, or pneumothorax.
b. Ddimer: is of limited value, because it can be increased in; pulmonary embolism,
myocardial infarction, pneumonia, and sepsis. If normal or less than 500n g/ml, then
PE can be excluded.
c. Troponin I and BNP are raised in case of right ventricular micro infarction.
d. CTPA is the 1st line diagnostic test. Used to diagnose; PE, consolidation,
pneumothorax, and aortic dissection. Sensitivity of CT can be increased by
simultaneous visualisation of the femoral and popliteal veins.CTPA is contraindicated
in patients with renal impairment and in patients with known allergy to iodinated
contrast.
e. Ventilationperfusion scanning is not widely used nowadays.
f. Colordoppler is used in patients with suspected DVT or pulmonary embolism.
g. ECG: is used in case of; massive PE, thrombus, LVF, aortic dissection, and pericardial
tamponade.
h. Pulmonary angiography is superseded by CTPA, but it is still useful in selected
patients where it is needed to deliver catheterbased therapies.
● Management:
a. Oxygen is given in hypoxemic patients. SaO2 goal is to be more than 90%.
b. IV fluids or plasma expander must be given in case of circulatory shock.
c. Inotropic agents are of limited value.
d. Diuretics and vasodilators should be avoided because they decrease the cardiac
output.
e. Opiates may be necessary to relieve pain and distress. They should be used
cautiously in hypotensive patients.
f. External cardiac massage may be successful to dislodge and break up a large central
embolus.
g. Anticoagulants: discussed below.
h. Thrombolytic therapy is always indicated in any patient with acute massive PE with
cardiogenic shock. In the absence of shock, the benefits are less clear. Thrombolytics
are also used in patients with right ventricular dilatation, hypokinesis, or severe
hypoxemia.
Scanning for hemorrhage risk should be done to avoid IC hemorrhage. Surgical
pulmonary embolectomy is used in high mortality patients.
i. Caval filters are of limited use, unless patient cannot be anticoagulated, patient
suffered a massive hemorrhage, or in case oF recurrent VTE despite the use of
anticoagulants.
● Anticoagulants:
○ Must be given in high or intermediate probability of PE.
○ In case of low probability, withhold the use of AC until further investigations are done.
○ Heparin:
■ Benefits:
● Reduces further propagation of clot.
● Decreases the risk for further emboli.
● Lowers mortality.
■ Given S/C and the dose is based on the weight.
■ It does not require monitoring tests of coagulation.
■ Fondaparinux, is a synthetic pentasaccharide and it is closely related to
heparin, and it is an alternative for LMWH.
○ Warfarin:
■ It is a vitamin K antagonist.
■ Needs about five days after administration to show its effect.
■ It requires continuous monitoring due to drug and food interaction, and due to
its narrow therapeutic index.
■ Thrombin or factor X inhibitors are preferred over warfarin because they do not
require coagulation monitoring.
○ LMWH S/C and oral warfarin are given simultaneously. After at least five days or when
the INR becomes 2 or more, stop LMWH because the effect of warfarin will start.
○ When to stop giving AC?
■ Patients with reversible risks→ after at least 3 months.
■ Patients with persistent risks for VTE and a history of previous emboli→ AC
given for life.
■ Patients with cancerassociated VTE (prostate, breast, and pelvic) → LMWH
for 6 months, then warfarin is given orally for life.
■ Patients with unprovoked VTE→ anticoagulation therapy for at least 3 months,
this duration must be prolonged in male patients with higher risks of recurrent
VTE. Other indications to increase the duration of therapy:
1. When the Ddimer is still high after one month of stopping AC.
2. Post thrombotic syndrome.
3. With those in whom recurrent PE may be fatal.
● Disadvantages of prolonged use of anticoagulants:
a. The inconvenience of longterm INR monitoring.
b. Major hemorrhage, lifethreatening hemorrhage, and fatal bleeding.
● Prognosis of VTE:
○ Very high mortality for those with ECG evidence of RV dysfunction or cardiogenic
shock.
○ Once AC is given→ there will be a rapid decrease in mortality.
○ Recurrence in the first 612 months is very high.
○ Recurrence within 10 years happens in ⅓ of the patients.