2. Definition
Cor pulmonale is defined as an alteration in the
structure and function of the right ventricle (RV)
of the heart caused by a primary disorder of the
respiratory system.
Right-sided ventricular disease caused by a
primary abnormality of the left side of the heart
or congenital heart disease is not considered cor
pulmonale.
3. Epidemiology
Cor pulmonale is estimated to account for 6-7% of all
types of adult heart disease in the United States.
chronic obstructive pulmonary disease (COPD) is the
causative factor in more than 50% of cases.
50,000 deaths in the United States are estimated to occur
per year from pulmonary emboli and about half occur
within the first hour due to acute right heart failure.
Globally, the incidence of cor pulmonale varies widely
among countries, depending on the prevalence of
cigarette smoking, air pollution, and other risk factors for
various lung diseases.
5. Pathophysiology
Common pathophysiologic mechanism is pulmonary
hypertension that is sufficient to alter RV structure (i.e.,
dilation with or without hypertrophy) and function.
parenchymal lung diseases, primary pulmonary vascular
disorders, or chronic (alveolar) hypoxia, the circulatory
bed undergoes varying degrees of vascular remodeling,
vasoconstriction, and destruction. As a result, pulmonary
artery pressures and RV afterload increase.
6. Pathophysiology
The response of the RV to pulmonary hypertension
depends on the acuteness and severity of the pressure
overload.
Acute cor pulmonale occurs after a sudden and severe
stimulus (e.g.massive pulmonary embolus), with RV
dilatation and failure but no RV hypertrophy.
Chronic cor pulmonale, however, is associated with a
more slowly evolving and progressive pulmonary
hypertension that leads to initial modest RV hypertrophy
and subsequent RV dilation.
7. Pathophysiology
systemic consequences of cor pulmonale relate to
alterations in cardiac output as well as salt and water
homeostasis.
Triggers include worsening hypoxia from any cause (e.g.,
pneumonia), acidemia (e.g., exacerbation of COPD),
acute pulmonary embolus, atrial tachyarrhythmia,
hypervolemia and mechanical ventilation that leads to
compressive forces on alveolar blood vessels.
13. Clinical Presentation
Signs
Tachycardia
Tachypnea
Elevated jugular venous pressures
Hepatomegaly
Lower extremity edema
On auscultation of the lungs, wheezes and crackles
Palpable P2, Left parasternal heave, splitting of S2, murmurs
of tricuspid regurgitation, pulmonic flow and regurgitation
(Graham Steele) murmurs.
14. Investigation
To see right ventricular dysfunction and
pulmonary hypertension
ECG
Echocardiography
Right heart catheterization
Cardiac magnetic resonance
15. Investigation
To identify the primary cause
Complete blood count – Polycythemia, increase
haematocrit.
Chest X-ray PA view
Pulmonary function test
Alpha 1- antitrypsin
ANA
anticentromere antibodies
Protein S and C, antithrombin III,
Arterial blood gas analysis
16. Investigation
Chest X-ray
Cardiomegaly
Enlargement of the central pulmonary arteries with oligemic peripheral lung fields.
Other finding regarding lungs disease.
Electocardiogram(ECG)
P pulmonale
right axis deviation
RV hypertrophy
Low-voltage QRS because of underlying COPD with hyperinflation.
2-D and Doppler Echocardiography
RV dilatation and/or hypertrophy and diminished function
Pulmonary hypertension.
21. Management of complication
Warfarin
Anticoagulation with warfarin is recommended in patients at high
risk for thromboembolism.
Recommended international normalized ratio range is 1.5 to 2.5
for PAH and cor pulmonale as prophylaxis.
Diuretics
Are used to decrease the elevated right ventricular (RV) filling
volume in patients with chronic cor pulmonale.
To manage RV volume overload, patients often respond well to a
combination of a loop diuretic, such as furosemide, and the
potassium-sparing diuretic spironolactone.
22. Surgical management
Phlebotomy is indicated in patients with chronic cor pulmonale and chronic
hypoxia causing severe polycythemia
Single-lung, double-lung, and heart-lung transplantation
23. Management of acute exacerbation
Hospitalization
Oxygen therapy
Control of heart failure
Control of cardiac arrhythmia
Control of infection
Treatment of lung disease accordingly
24. Prognosis
The prognosis of cor pulmonale is variable depending upon
the underlying pathology.
Development of cor pulmonale as a result of a primary
pulmonary disease usually poor prognosis.