Pulmonary embolism: An

enemy that we often neglect

Dr. Md. Abu Bakar Siddique Majumdar
Dept. of Cardiology
Dhaka Medical College Hospital
Introduction
• Despite high incidence of pulmonary embolism its diagnosis is difficult, primarily
due to vagaries of symptoms and signs at presentation.

• Pulmonary embolism needs high clinical suspicion and aggressive treatment to

prevent mortality and morbidity.

• We encountered two cases of acute breathlessness who later diagnosed as a case of

pulmonary embolism due to inherited thrombophilia.
Case 1

• A 32-year-old male banker presented in CCU with

ØH/O progressive SOB for 1 month and

ØRecurrent syncope for 3days.

• Patient was hemodynamically unstable with heart rate 130 bpm regular, BP 80/60

mmHg.
Case1cont..
• ECG showed
Ø sinus tachycardia
Ø complete RBBB
Ø S1, Q3, T3 pattern

• Echocardiography- dilated RA and RV. RV systolic dysfunction with Mcconnell’s

sign.

• Duplex of lower limb vessels showed no abnormality. D-Dimer was very high

(8214 ng/ml). S Troponin I was upper limit of normal level.

Case1cont..
• On suspicion of PE, we promptly started UFH but patient didn’t improve.

• On next day, CTPA was done which confirm massive pulmonary embolism. PESI
was 112 which indicates high risk of mortality.

• Tenecteplase 35mg single bolus dose was given with switching to Enoxaparin.
From next day patient improved clinically.
Follow up
• The patient was discharged in good general status with Apixaban 10mg BD for
7days followed by 5mg BD.

• After 1month thrombophilia scan were advised which showed Protein S

deficiency.
 ECG before and after treatment
Sinus tachycardia, Incomplete RBBB, S1Q3T3 Enlarged RA, RV strain
pattern, enlarged RA
 CTPA before and after treatment
1. Large PE with presence of emboli in main
RPA and main LPA and segmental PA.
2. Few small thrombus in pulmonary trunk
and RV and IVC
1. Thrombus in RPA and LPA and peripheral
branches.
2. MPA and cardiac chambers are free of
thrombus
Thrombophilia scan
Result Normal range
Protein C 102% 70-130 %
Protein S 31% 60-130 %
Fibrinogen 329 mg/dl 200-400 mg/dl
Factor V 66.5% 70-120 %
Anti-Thrombin III 72.8% 80-120 %
Final diagnosis

Acute massive pulmonary embolism due to Protein S deficiency

Case 2
• A 20-year old- female presented in CCU with
Ø dyspnea and cough for 5days.

Ø H/O NVD 1 month back.

• On examination she was hemodynamically stable with

Ø Raised JVP

Ø Left parasternal heave and

Ø Loud P2.
Case 2 cont..
• ECG was normal

• Echocardiography showed
Ø dilated RA, RV with RV dysfunction and normal LV function

Ø No shunt anomaly

• D-Dimer level was high. PT, APTT, renal and liver function was normal. Duplex
of lower limb vessels were normal
Case 2 cont..
• CTPA was done which showed Thrombus in both pulmonary artery with complete
occlusion of left lower pulmonary artery.

• Screening for connective tissue disease was done and it revealed-

Connective tissue disease screening
Result Normal range
ANA 4.07 >1.2
Anti dsDNA 210 U/ml >75 U/ml
Anti phospholipid Ab IgG- 2.55 U/ml (negative) >10 U/ml positive
IgM- 1.95 U/ml (negative) >10 U/ml positive
Anti Cardiolipin Ab IgG- 1.98 U/ml (negative) >15 U/ml positive
IgM- 2.10 U/ml (negative) >15 U/ml positive
Anti Ro Ab Positive
Anti La Ab Positive
Urine for PCR Negative
Follow up
• We started warfarin and INR was monitored.

• After 3 months follow up-

Ø Right heart function was improved and

Ø Repeat CTPA showed regression of thrombus in both pulmonary arteries.

• Anticoagulant was stopped for 2weeks and blood sample was sent for
thrombophilia scan which yielded-
Thrombophilia scan
Result Normal value
Protein C 18.4% 70-130%
Protein S 24% 50-120%
Antithrombin III 90.2% 80-120%
Factor V 61.2% 70-120%
Fibrinogen 259 mg/L 200-400 mg/L
Final diagnosis

So, our final diagnosis changed to- SLE with protein C and Protein S
deficiency
Discussion
• Proteins C and S deficiency are associated with high risk of thrombosis.

• In patients presenting with VTE approximately 3-5% had protein C and protein S
deficiency.

• It can also cause ischemia in various organs such as heart and brain.
Mechanism of Protein C and protein S
Protein C
• Protein C is a vitamin K dependent glycoprotein primarily synthesized in Liver
and circulates in an inactive form.
• Heterozygous protein C deficiency is inherited in an autosomal dominant fashion.
• The gene for protein C is located on the long arm of chromosome 2.
• Nearly 200 pathogenic mutations have been described. These mutations are
divided into 2 types-
• Type I (Quantitative) and
• Type II (Qualitative)
Protein C cont..
• Approximately 40% of patients with VTE have one of the risk factors, such as
pregnancy, the postpartum state, hormonal therapy, surgery, or immobilization,
COVID 19 infection

• Approximately 40% of patients with protein C deficiency present with pulmonary

embolism, and roughly 60% suffer recurrent thrombosis without anticoagulation.

• Patients with levels

Ø <50%- true hereditary deficiency,
Ø 55% to 65%- heterozygous deficiency.
Protein S
• Protein S was named after Seattle, Washington, where it was first discovered and
purified in 1979.

• PS deficiency inherited in an autosomal dominant fashion with mutations in

the PROS1 gene on chromosome 3.
Protein S cont..
• It has two forms-
• Free form
• Total (Bound with C4BP)

• protein S deficiency was classified into the following three phenotypes-

Ø Type I deficiency Both total and free PS antigen are reduced (quantitative deficiency)

Ø Type II deficiency functional deficiency (qualitative defect)

Ø Type III deficiency low free protein S levels with normal total protein S
Acquired causes of Protein C and Protein S
deficiency

• Oral contraceptive use • Certain viral infections (eg, HIV,

• Warfarin anticoagulant use varicella)

• Nephrotic syndrome • Systemic lupus erythematosus

• Myeloproliferative disorders
• Vitamin K deficiency
• Chronic liver disease • COVID-19
Take home message
• Pulmonary embolism isn’t rare, it is commonly missed.

• Pulmonary embolism needed high clinical suspicion to diagnose. If we miss,

mortality rate is very high.

• Risk factor detection is very important to treat these cases.

• Aggressive management and regular follow up can give patient a new life.
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