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Acute
Cor Pulmonale (Pulmonary Embolism)
Author:
Musab T. Ejami, MBBS, Faculty Of medicine, University Of Al-Zaiem Al-Azhari
Two words best characterize the mortality
and morbidity of due to venous thromboembolism: substantial
and unacceptable.
K.M. Mosers. Am. Rev. Resp. Dis.
1990
Introduction:
The term “Cor Pulmonale” means right heart failure arising
secondary to pulmonary hypertension. However the term is
unsatisfactory in that it is not a distinct disease, but
represents very different disease process, so it should be
remembered that is actually a final common pathway for the
courses of these diseases not a distinct one. Pure right
heart failure is uncommon, though it may occur following:
* Right ventricular myocardial infraction.
* Isolated right-sided valvular disease.
As said the causes of cor-pulmonale are numerous, but in
clinical practice, one disorder of each type predominates:
* Pulmonary embolism as a cause for acute cor-pulmonale.
* COPD for chronic cor-pulmonale.
PE: Background:
Pulmonary embolism (PE) is a common and potentially lethal
disease; unfortunately, the diagnosis is often missed
because PE presents with nonspecific signs and symptoms. If
left untreated, approximately one third of patients who
survive an initial PE subsequently die from a future embolic
episode. Most patients succumb to PE within the first few
hours of the event. In patients who do survive, recurrent
embolism and death can be prevented with prompt diagnosis
and therapy.
Epidemiology:
* Unexpected death from a massive PE is second only to the
sudden cardiac death. Autopsy studies of hospitalized
patients have shown approximately 80% of these patients died
from massive PE.
* Approximately 10% of patients who develop PE die within
the first hour, and 30% die subsequently from recurrent
embolism. Anticoagulant treatment decreases the mortality
rate to less than 5%.
* The diagnosis of PE is missed in approximately 400,000
patients in the United States per year; approximately
100,000 deaths could be prevented with proper diagnosis and
treatment.
Pathophysiology:
Sources of
Emboli:
Veins of lower limb, ileo-femoral veins and calf veins may
be the source, rarely emboli originating from the right
atrium in atrial fibrillation. The risk factors are the same
for those of DVT.
Respiratory consequences
Acute respiratory consequences of PE include increased
alveolar dead space, pneumoconstriction, hypoxemia, and
hyperventilation. Later, 2 additional consequences may
occur: regional loss of surfactant and pulmonary infarction.
Arterial hypoxemia is a frequent but not universal finding
in patients with acute embolism. The mechanisms of hypoxemia
include ventilation-perfusion mismatch, intrapulmonary
shunts, reduced cardiac output, and intracardiac shunt via
patent foramen ovale. Pulmonary infarction is an uncommon
consequence because of the bronchial arterial collateral
circulation.
Hemodynamic consequences
PE reduces the cross-sectional area of the pulmonary
vascular bed, resulting in an increment in pulmonary
vascular resistance, which, in turn, increases the right
ventricular afterload. If the afterload is increased
severely, right ventricular failure may ensue. In addition,
the humoral and reflex mechanisms contribute to the
pulmonary arterial constriction. Prior poor cardiopulmonary
status of the patient is an important factor leading to
hemodynamic collapse.
Clinical Presentation:
Many pulmonary embolisms occur silently, yet there are three
typical clinical presentations, which are:
* Small/medium pulmonary embolism
* Massive pulmonary embolism
* Multiple recurrent pulmonary emboli.
1- Small/Medium Pulmonary
Embolism:
In this case the embolus has impacted in the terminal
pulmonary vessel. Symptoms are:
* Pluritic chest pain.
* Breathlessness.
* Haemoptysis. (30% of cases, 3 -5 days after the initial
event).
Physical examination may reveal a pyrecxial/ tachypnic
patient with the following:
* Pleural rub.
* Coarse crackles.
* Pleural effusion.
Differential diagnosis:
2- Massive Pulmonary Embolism:
It occurs due to sudden obstruction of the right ventricular
outflow tract. The patient has the following symptoms:
* Severe central chest pain (due to cardiac ischemia).
* Syncope and collapse.
Clinical examination may reveal some or all of the
following:
* Tachypnic and tachycardic with hypotension and peripheral
shutdown.
* Raised JVP with prominent “a” wave.
* Positive left Parastenral heave.
* Gallop rhythm.
* Palpable 2nd heart sound.
* Wide splitting of the 2nd heart sound.
* Pansystolic murmur over the left sternal edge may be noted
(due to tricuspid regurgitation).
Differential Diagnosis:
myocardial infarction, septic shock and hypovolemia.
3- Multiple recurrent emboli:
This occurs due to multiple occlusions of the pulmonary
vasculature by small emboli, thus these patients do not
present acutely, but run a more chronic course with the
gradual development of pulmonary hypertension, and symptoms
are:
* Increasing breathlessness over weeks or months.
* Weakness and syncope on exertion.
* Occasional angina.
Physical examination may reveal Signs of pulmonary
hypertension (palpable 2nd heart sound, loud 2nd heart
sound, enhanced splitting, Raised JVP and left a left
parasternal heave). Signs of established of right-sided
heart failure may be present.
Differential diagnosis:
CHF and asthma.
Index of Suspicion:
The symptoms of PE are nonspecific; therefore, a high index
of suspicion is required, particularly when a patient has
risk factors.
The most common symptoms of PE in the Prospective
Investigation of Pulmonary Embolism Diagnosis (PIOPED) study
were:
* Dyspnea (73%)
* Pleuritic chest pain (66%)
* Cough (37%)
* Hemoptysis (13%).
The most common physical signs in the PIOPED study were as
follows:
* Tachypnea (70%)
* Rales (51%)
* Tachycardia (30%)
* Fourth heart sound (24%)
* Accentuated pulmonic component of the second heart sound
(23%)
* Fever of less than 39°C may be present in 14% of patients;
however, temperature higher than 39.5°C is not from PE.
* Chest wall tenderness upon palpation, without a history of
trauma, may be the sole physical finding in rare cases
Investigations:
Clinical signs and symptoms are nonspecific; therefore,
patients suspected to have PE must undergo diagnostic tests
until the diagnosis is ascertained or eliminated or an
alternative diagnosis is confirmed.
Blood Test:
If pulmonary infarction has occurred there will be a
polymophonuclear leuckocytosis, elevated ERS and increased
serum LDH.
Blood Gases:
May show hypoxia and hypocapnia in massive pulmonary
embolism..
Plasma D-Dimer:
D-dimer, a degradation product produced by plasmin-mediated
proteases of cross-linked fibrin is detected in over 90% of
the cases, however false positive may be as high as 70%.
Chest X-ray:
Small/Medium:
Often normal, but
the following features can be found:
- Linear atelectasis.
- Blunting of costophrenic angle.
- Raised hemidiaghram
-Wedge-shaped pulmonary infarct.
- Cut-off sign.
Massive:
- Oligaemia
- Dilatation of the pulmonary artery at the hilia
- Often no changes.
Recurrent:
May be normal,
enlarged pulmonary arterioles with oligaemic zones usually
indicate advanced disease.
Electrocardiogram (ECG):
Small/Medium Emboli:
The ECG is usually normal, except for signs tachycardia. But
the following also may occur:
- Atrial fibrillation.
- Other forms of tachycardia.
- Evidence of right ventricular strain.
Massive Embolism (see Figure
3):
* incomplete/complete RBBB
* S wave in lead I and aVL > 1.5 mm (or r:s ratio < 1)
* clockwise rotation of the heart and a shift in the
transitional zone to lead V5 or further
* Q waves in leads III and/or aVF
* frontal plane axis to the right of + 90 degrees (or an
indeterminate axis)
* low voltage (< 5 mmm) QRS complexes in the limb leads
* T wave inversions in lead 3, aVF and/or leads V1 - 4
* elevated ST segments in leads aVR and V1 - 2
* prominent P waves in leads II, III and aVF
* S1,Q3, T3 pattern or S1,S2,S3 pattern
* ischemic appearing ST segment/T wave changes in the
inferior + anteroseptal leads
* cardiac arrythmias (sinus tachycardia, atrial flutter,
atrial fibrillation)
Multiple Recurrent emboli:
Normal or show signs of pulmonary hypertension:
- Right axis deviation.
- Dominant R waves in V1.
- Prolongation of QRS complex.

Previous Figure:
ECG abnormalities include:
* Prominent P waves (P Pulmonale) in leads II, III and aVF
suggesting right atrial enlargement/strain
* a prominent R wave in lead aVR
* Borderline ST elevation in lead aVR
* disproportionately large s waves in the limb leads with a
r/s ratio < 1 in lead I and aVL
* RS complexes in the precordial leads which extend all the
way to lead V5
* Small QRS complexes in the limb leads
* An indeterminate frontal plane axis.

Radionucleotide Ventilation
Perfusion Scan (V/Q):
A very good and important diagnostic tool, 99mTc scintigram
shows the unperfused area in the lung and if accompanied by
a normal CXR or ventilation scan, then PE is highly
suggested. The PIOPED classification scheme allows the
interpretation of the V/Q scan more meaningfully:
Diagnostic pattern - Normal V/Q scan findings or
findings indicating a high probability:
* Normal V/Q scan findings indicate an absence of any
perfusion defects. Four percent of these patients still may
have PE. Unless the patient has features indicating very
high clinical suspicion, these findings may be considered
negative for PE.
* High-probability scan findings are 2 or more segmental or
1 larger perfusion defect in the presence of normal chest
radiography findings and ventilation scan findings.
Approximately 87% of these patients were found to have PE.
If accompanied by high pretest probability, the likelihood
of pulmonary emboli increases to 95%.
Diagnostic pattern - Nondiagnostic scans
interpreted as low or intermediate probability:
* Low-probability scan findings consist of small perfusion
defects associated with corresponding abnormalities upon
chest radiograph or ventilation scan. A single segmental
perfusion defect with normal chest x-ray findings and
nonsegmental perfusion defects are common nondiagnostic
patterns. Twelve percent of the patients with this pattern
have PE, unless the patient has a very low pretest
probability or clinical suspicion. Further diagnostic
studies must be carried out to confirm or exclude the
diagnosis of PE.
* Intermediate probability is indicated by those patients
with any V/Q abnormality that is not classified as high or
low probability. Approximately 30% have PE; therefore, the
finding of this scan pattern must be followed by further
investigation to definitely exclude the diagnosis of PE.
Pulmonary Angiography:
The golden standard diagnostic investigation for PE,
angiographic signs include cut-off sign due to intra-luminal
defect and poor perfusion in supplied areas.

Echocardiography:
It is not specific, and of no diagnostic value, it shows
vigoursly contracting left ventricle & occasionally a clot
in the right ventricle outflow tract with strain compatible
with pulmonary hypertension. Transesophageal
echocardiography may identify central PE, and the
sensitivity for central PE is reported to be 82%
Ultrasound:
Detection of clots in the pelvic or femoral veins.
Spiral CT-scan:
With intravenous contrast, show god sensitivity to medium
pulmonary emboli, they do not exclude small emboli.
MR imaging:
Similar results to CT.
Management of
Thromboembolism:
Management For established
embolism:
1- Acute Management:
* Admit the Patient to ICU.
* Oxygen by mask 60-100% unless COPD.
* Analgesia.
* I.V. Fluids & intropic agents if haemodynamiclly
collapsed.
2- Anticoagulants:
* Aim: prevention of further emboli.
* Unfractioned Heparin 5000U – 10,000 I.V. Bolus is given
Followed by infusion at a rate of 1000 U/h for 24 hours.
* Warfarin is introduced on day 5 in a dose of 10mg/day for
2 days and later dosage adjusted to keep PT more than 1.5 –
2 times control for a period of 3 month.
* LMWH although more expensive are increasingly being used,
that is because they simplify the treatment.
* Risk of Heparin: ineffectiveness due to in sufficient dose
bleeding and thrombocytopenia.
3- Thrombolytic Therapy:
* Aim: Dissolution of emboli.
* Indications: Massive PE with hypotension, cardiopulmonary
failure with shock, angiographic evidence of occlusion of
40% of the pulmonary circulation.
* 250,000 U streptokinase I.V. infusion over 30 min,
followed by 100,000 untis I.V. hourly.
* Other agents include: Urokinase – loading dose 4400 U over
10 min, infusion 4400u/kg for next 12 hours. Or tPA: 100mg
infused over 24 hours.
Prevention Of thrombo-embolism:
* An objective for critically ill in high risk group.
* Modify Modifiable Risk factors such as smoking, obesity,
pregnancy, oral contraceptives.
* 5000 u 12hrly is effective, LMW heparin 3000us/e OD is a
good prophylaxis.
* Caval filters, compression stockings and other means of
preventing DVT should be undertaken.
References & Resources:
1. Parveen Kumar & Michael Clark: Clinical Medicine 4th
edition.
2. Pulmonary embolism: Sat Sharma, MD, FRCPC, FCCP, DABSM,
Program Director, Associate Professor, Department of
Internal Medicine, Divisions of Pulmonary and Critical Care
Medicine, University of Manitoba; Site Coordinator of
Respiratory Medicine, St Boniface General Hospital (article
at: www.emedicine.com)
3. Dr. Shyam Sunder, MD: Current Status in diagnosis,
management and prophylaxis in pulmonary embolism.
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