Cor pulmonale is a condition where the right ventricle of the heart enlarges and fails due to long-standing increased workload from diseases that affect the lungs like COPD. It is defined as hypertrophy and dilation of the right ventricle resulting from increased pulmonary vascular resistance. The main causes are chronic hypoxemia and pulmonary thromboembolism which lead to remodeling of the pulmonary arteries and increased pressure in the lungs. Over time, this puts strain on the right ventricle and can cause it to fail. Treatment focuses on reducing pulmonary pressures through oxygen therapy and vasodilators while managing symptoms of right heart failure.

1. ‫بسم ال الرحمن الرحيم‬
Cor pulmonale
By
Prof. Dr. Rasheed Abd El Khalek
.M. D
Head Of Internal Medicine & Intensive Care
Department

3. Definitions
 Cor pulmonale is a latin word means ”pulmonary heart” .
 The world Health Organizaton in 1963 adopted this definition of
cor pulmonale :” hypertrophy of the right ventricle resulting from
diseases affecting the function and/or structure of the lungs,
except when these pulmonary alterations are the result of
diseases that primarily affect the left side of the heart , as
congenital heart diseases”
 Cor pulmonale is a disease of the right ventricle characterized by
hypertrophy and dilation that results from diseases directly
affecting the lung parenchyma or lung vasculature. Of note ,right
heart failure need not be present in cor pulmonale .

4. Subtypes Of Cor Pulmonale
 Cor pulmonale can be either acute or chronic in development.
 Acute cor pulmonale is the result of a sudden increase in right
ventricular pressure, as seen in massive pulmonary embolism or
acute respiratory distress syndrome.
 Chronic cor pulmonale can be further characterized by hypoxic or
vascular obliterans pathophysiology.
 The most common disease process associated with hypoxic
subtype is chronic obstructive pulmonary disease (COPD).
 The most common process associated with obliterans subtype is
pulmonary thromboembolic disease.

5. Pathophysiology
 Under normal phsyologic conditions, the right ventricle pumps against a low-
resistance circuit.
 Normal pulmonary vascular resistance is approximately one-tenth the
resistance of the systemic arteries.
 The right ventricle is thin walled and able to accommodate considerable
changes in volume without large changes in pressure.
 Increased cardiac output leads to recruitment of underperfused pulmonary
vessels and distention of other pulmonary vessels.
 The initial pathophysiologic event in the production of cor pulmonale is
elevation of the pulmonary vascular resistance.
 As the resistance increases, the pulmonary arterial pressure rises, right
ventricular work increases, right ventricular hypertrophy (i.e.,thickening,
dilation, or both ).
 Right ventricular failure occurs when compensation through dilation and
hypertrophy are exhausted.

6. Causes
 Any process that results in pulmonary
hypertension can cause cor pulmonale.
 Pulmonary hypertension is defined as
mean pulmonary artery pressure >20
mmHg at rest or >30 mmHg with
exercise.

8. Pathophysiology of pulmonary
hypertension
1. Hypoxic pulmonary vasoconstriction and
arterial occlusion are the major causes of
pulmonary hypertension.
2. Both produce reduced blood flow with
increased vascular resistance.
3. Acute hypoxic pulmonary vaso –constriction
optimizes ventilation – perfusion relationships
when regional ventilation demands in the lung
are not met.

9. 4. However, chronic hypoxemia leading to
chronic vasoconstriction produces
smooth ms proliferation in small
pulmonary arteries.
5. Decreased luminal cross sectional
diameter leads to increased resistance
and increased pulmonary artery
pressure.

10. 6. These architectural changes in
pulmonary arteries may promote platelet
aggregation and activation.
7. This leads to thrombi formation that
further increases pulmonary vascular
resistance and pulmonary hypertension.

11. 8. Hypoxemia produces changes in
vascular mediators such as Nitric Oxide,
Endothelin1 (ET1) and platelet derived
growth factors (PDGf A and B).
9. Nitric oxide is a vasodilator; hypoxemia
reduces endothelial cell production of
nitric oxide and results in impaired
smooth ms relaxation.

12. 10. Hypoxemia increases ET1 production
and PDGF A and B .
11. ET1 is apotent vasoconstrictor, and
PDGF A and B results in pulmonary
vascular remodeling.
12. All causes increased pulmonary artery
resistance and causes pulmonary
hypertension.

13. Presentation
 The signs and symptoms of cor
pulmonale are often subtle unless the
disease process becomes far advanced.
 In addition, clinicians tend to focus on
the disease giving rise to cor pulmonale
rather than on cor pulmonale itself.
 Manifestations of cor pulmonale are
similar to those of right side heart failure.

14. :Symptoms of Cor Pulmonale
 Fatigability
 Dyspnea on exertion
 Syncope
 Chest pain
 Palpitation
 Abdominal edema or distension
 Lower extremity edema

15. Clinical Signs Of Cor
:Pulmonale
 Accentuated A wave of the jugular venous
pulsations
 Prominent jugular V wave, indicating the
presence of tricuspid regurgitation
 Palpable left parasternal lift
 Accentuated pulmonic component of the
second heart sound
 Right sided S4 heart sound

16.  Murmurs of tricuspid and pulmonic
insufficiency
 Dependent prepheral edema and
hepatomegaly

17. The mortality associated with cor
pulmonale
 Patients with COPD have a 60% 5-year survival
rate, whereas patients with COPD and
pulmonary artery pressure in excessof 25 mmHg
have a survival of only 36%.
 The 5-year survival rate for patients with COPD
who develop preipheral edema is approximately
30%.
 It is unclear whether pulmonary artery
hypertension is the cause of death or whether it
is a marker of increased motality.

18. Electrocardiography criteria of right
ventricular hypertrophy
 Right axis deviation.
 P pulmonale (large P wave ) in the inferior
and anterior leads “ right atrial
enlargement “.
 Right bundle branch block.
 Right precordial T-wave inversions.
 Delayed interinsicoid deflection of right
precordial leads.

19. E.C.G criteria of R.V.H
 S1Q3 T3 pattern.
 QR pattern in lead V1 or V3R.
 An R wave in V1 or V3R.
 An R/S ratio >1 in V1 or <1 in V5 or V6.

20. S1 Q3 T3 Pattern

21. Investigations Help In Diagnosis
 Chest Radiograph:
1. Enlarged pulmonary artery.
2. Enlarged right ventricle.
3. Distended azygous or other central vein.
4. Westermark sign “oligemia of lung lobe or
entire lung “.
5. Hampton’s hump “wedge shaped opacity
6. COPD signs as anterior-posterior diameter ,
flattening of diaphragm , honeycombing and
hyperlucency.

23.  Computed tomography:
1. Main pulmonary artery diameter
measurements >29 mm have a
sensitivity of 84 % and specificity of 75%
for the diagnosis of pulmonary
hypertension.
2. There are data to suggest that an
enlarged main pulmonary artery
diameter and ratio of segmental
pulmonary artery diameter to
corresponding bronchus diameter > 1
increases the specificity of a pulmonary
hypertension diagnosis.

24.  Echocardiography :
1. An adequate examination is reported in
up to 65 – 80 % of patients with COPD
because of the technical difficulty
associated with hyperinflation.
2. A better examination can be obtained
with transesophageal echocardiography.
3. Doppler echocardiography has aided in
the assessment of pulmonary artery
pressure by measuring the flow of
regurgitant blood across the tricuspid
valave or by measuring right vetricular
ejection flow.

26.  Right heart catheterization :
1. This is the gold standard for thorough
evaluation and diagnosis of pulmonary
hypertension.
 Radionuclide angiography (gated blood
pool scan ):
1. This test is most useful for measuring right and
left ventricular ejection fraction.
 Magnetic resonance imaging :
1. This non invasive technique yields highly
accurate dimensions of the right ventricle.

27. Treatment
 Non pharmacological treatment :
1. Oxygen therapy.
2. Phlebotomy.
3. Non invasive positive pressure ventilation
(NIPPV).
 Pharmacological treatment :
1. Diuretics .
2. Anticoagulation .
3. Vasodilators .

28. Oxygen therapy
 This is considered a mainstay of treatment for
patients with COPD .
 Large controlled trials demonstrate that long
term administration of oxygen improves survival
in hypoxemic patients with COPD.
 Oxygen therapy decreases pulmonary vascular
resistance by diminishing pulmonary
vasoconstriction and improves right ventricular
stroke volume and cardiac output .

29. Phlebotomy
 In patient with pronounced polycythemia
(hematocrit >60 % ), phlebotomy may provide
symptomatic relief .
 In resting patients , phlebotomy can affect a mild
decrease in pulmonary artery pressure and
pulmonary vascular resistance .
 In general , blood viscosity has less effect than
blood volume on pulmonary arterial pressure .
 Phlebotomy , with a goal hematocrit of 50 %,
may improve exercise tolerance in patients with
polycythemic COPD.

30.  Phlebotomy is not an optimal single
therapy but can be considered in
polycythemic patients with acute
decompensation .

31. Noninvasive positive pressure
(ventilation ( NIPPV
 For patients with acute COPD
exacerbations, NIPPV has been shown to
improve outcomes in acute hospitalization.
 No such data exist for long-term treatment
of COPD or sleep-disordered breathing
with NIPPV.
 There is evidence that oxygenation is
improved in these patients, but reduction
in pulmonary artery pressure is only
anecdotal.

32. Diuretics
 Diuretic therapy with salt restricted diet
may be needed in congestive heart failure
to take care of the excessive water that
the lungs share and to improve alveolar
ventilation and gas exchange.
 However, the use of diuretics may
produce hemodynamic adverse effects,
such as volume depletion, decrease
venous return to the right ventricle, and
decreased cardiac output.

33.  Another complication is the production of
hypokalemic metabolic alkalosis, which
diminishes the Co2 stimulus to the
respiratory center, decreasing ventilatory
drive.

34. Anticoagulation
 Chronic anticoagulation with Warfarin may
provide benefit for those patients with Cor
Pulmonale resulting from thrombo-
occlusive pulmonary disease.

35. vasodilators
 Vasodilators improve cardiac output in
many patients with cor pulmonale.
 However, treatment with vasodilators may
be associated with adverse effects,
including systemic hypotension that
coronary perfusion pressure, blunting of
hypoxic pulmonary vasoconstriction and
circulatory collapse.

36. Different classes of vasodilators
used in Cor Pulmonale
 Nonspecific vasodilators:
1. Hydralazine increases cardiac output in
patients with COPD; however, its ability
to decrease pulmonary artery pressure is
unpredictable.
2. Nitroprusside may provide benefit but
also runs the risk of systemic
hypotension and compromise of
adequate coronary perfusion pressure.

37. 3. Calcium channel blockers such as
Nifedipine reduce pulmonary vascular
resistance and increase cardiac output
only for the short term.
4. Verapamil and Diltiazem have not
proved effective in dilating pulmonary
vasculature.

38.  Pulmonary vasodilators :
1. Prostaglandins decrease pulmonary
artery pressure and increase right
ventricular ejection fraction and cardiac
output.
2. Aerosolized prostacyclin causes
pulmonary artery vasodilatation and
improves cardiac output and arterial
oxyhemoglobin saturation in patients
with chronic pulmonary hypertension.

39. 3. Nitric oxide provides a real clinical
scenario. It reliably decreases pulmonary
vascular resistance without causing
systemic hypotension and preserves or
improves optimal ventilation-perfusion
match. Its drawbacks are difficult
administration, high cost, and a well-
documented tachyphylactic effect.
Multiple studies have shown that its
benefits are most significant for only 1-3
days, especially in patients with acute
respiratory distress syndrome.

40.  Inotropes with vasodilatory
properties :
1. Dobutamine is an inotropic agent with
vasodilatory effect which improves right
ventricular function and cardiac output,
but its effect on systemic blood pressure
is unpredictable.
2. Amrinone lowers pulmonary artery
pressure and rises cardiac output and
systemic blood pressure.

41.  Endothelin receptor antagonist :
1. Bosentan is an endothelin receptor
antagonist that produces pulmonary
vasodilation and attenuates ventricular
remodeling and improve survival on
chronic use.

42. Role of digoxin in treatment
 Cardiac output improves in about 10% of
patients with primary pulmonary
hypertension who receive digoxin.
 This rate is similar to that in patient with
left ventricular dyfunction.
 Patients who receive digoxin also show a
modest increase in pulmonary pressure,
perhaps due to increase in cardiac output.

43.  Clinical studies show improvement in right
ventricular function only in those patients
who have reduced left ventricular ejection
fraction.
 Recently, digoxin has fallen out of favor in
the setting of left ventricular dysfunction;
the trend in clinical medicine has been its
continued use in rate control.

44. KEY POINTS OF COR
PULMONALE
 Right side heart failure is not necessary to make
the diagnosis of cor pulmonale.
 Any process that cause pulmonary hypertension
can cause cor pulmonale.
 COPD is the most common cause of chronic cor
pulmonale.
 Cor pulmonale is common in advanced
obstructive lung disease and has a poor 5-year
survival rate.
 Ventricular interdependence can developin late
stages of cor pulmonale.

45. PRAISE BE TO ALLAAH
THANKS