1. Department of Medicine Manipal College of Medical Sciences
Cor pulmonale
ALOK SINHA
Department of Medicine
Manipal College of Medical Sciences
Pokhara, Nepal

2. Right sided heart disease secondary to lung disease
Cor pulmonale is defined as an alteration in the structure and function of the right ventricle caused by a primary disorder of the respiratory system – lung parenchyma, lung vasculature or thoracic cage
Right sided heart disease secondary to lung disease
Pulmonary hypertension is the common link between lung dysfunction and the heart in cor pulmonale .

3. Pathophysiology 1.Pulmonary vasoconstriction due to a.Hypoxia
b.Blood acidemia
2. Obliteration of the pulmonary vascular bed secondary to lung disorders –
a. emphysema
b. pulmonary thrombo embolism
c. interstitial lung disease

4. 3. increased blood viscosity secondary to blood disorders
polycythemia vera
sickle cell disease
macroglobulinemia
4. idiopathic primary pulmonary hypertension

5. decrease L.V. volume – decresed coronary
Pul art pressure – dilatation of R V – reduced C O & septal displacement--
decrease L.V. volume – decresed coronary
blood to R V – further detoriation of R V
function
Septum pushed to left
Reversed Bernmeim’s effect

6. patient with acute pulmonary hypertension
due to pulmonary embolism
After clot lysis

7. Acute cor pulmonale a. massive pulmonary embolism (more common)
b. acute respiratory distress syndrome (ARDS).
is associated with R V dilatation

8. Chronic cor pulmonale
C O P D > 50% of cases

9. Disorders with primary involvement of pulmonary vasculature and circulation
Repeated pulmonary emboli
Pulmonary vasculitis
Pulmonary veno-occlusive disease
Sickle cell disease
High altitude disease with pulmonary vasoconstriction
Primary pulmonary hypertension

10. Disorders with secondary involvement of pulmonary vasculature and circulation
Parenchymal lung diseases
Chronic obstructive pulmonary diseases
interstitial lung diseases
Neuromuscular disorders
myasthenia gravis
Poliomyelitis
amyotrophic lateral sclerosis
Obstructive and central sleep apnea
Thoracic deformities
Kyphoscoliosis
Ankylosing spondylitis

11. CLINICAL FEATURES

12. Clinical manifestations of cor pulmonale nonspecific
symptoms subtle in early stages of the disease mistakenly attributed to underlying pulmonary pathology which are:
Easy fatigability
Tachypnea
Exertional dyspnea
Cough
Followed by

13. because of rupture of a dilated or atherosclerotic pulmonary artery
1. Anginal chest pain
Right ventricular ischemia (does not respond to nitrates)
Rt. coronary artery stretching in dilated A-V groove following RVH
2. Hemoptysis
because of rupture of a dilated or atherosclerotic pulmonary artery

14. 3. A variety of neurologic symptoms may be seen due to decreased cardiac output and hypoxemia
impaired cognitive & higher mental functions

15. the left recurrent laryngeal nerve by a dilated pulmonary artery
4. Rarely hoarseness due to compression of
the left recurrent laryngeal nerve by a dilated pulmonary artery
5. In advanced stages, passive hepatic congestion secondary to severe right ventricular failure lead to
anorexia
right upper quadrant abdominal discomfort
jaundice

16. 6. Syncope with exertion seen in severe disease
reflects a relative inability to increase cardiac output during exercise with a subsequent drop in the systemic arterial pressure
7. Peripheral edema

17. Physical findings

18. may reflect
a. The underlying lung disease
b. pulmonary hypertension
c. RVH
d. RV failure

19. On inspection
1.An increase in chest diameter
2.Laboured respiratory efforts with retractions of chest wall
3.distended neck veins with prominent “a” or giant “v” waves
4.cyanosis may be seen

20. RVH - characterized by
Epigastric pulsation
left parasternal heave
Apex beat: in 5th ICS outside MCL diffuse, ill suatained
+ Hepatojugular reflex and pulsatile liver are signs of RV failure with systemic venous congestion
On percussion, hyper resonance of the lungs may be a sign of underlying COPD
ascites seen in severe disease

21. On auscultation of the chest
wheezes & crackles: signs of underlying lung disease
in early stages
1. Splitting of the S2
2. Loud P2

22. in advanced disease 2. Followed by ejection systolic murmur
1. sharp ejection click (single or multiple) over the pulmonary artery
2. Followed by ejection systolic murmur
3. Latter on: diastolic pulmonary regurgitation murmur (Graham steel)
4. may be S3 &/or S4
5. systolic murmur of tricuspid regurgitation

23. DIFFERENTIAL DIAGNOSIS
Congestive (biventricular) heart failure
Primary pulmonic stenosis
Primary pulmonary hypertension
Right-sided heart failure due to congenital heart diseases
Right heart failure due to right ventricular infarction

24. INVESTIGATIONS

25. Routine investigation:
Hematocrit
> 50 polycythemia
> 60 – indication for phlebotomy

26. To confirm diagnosis E C G X ray chest Echocardiography
Right heart catheterization

27. E C G in Cor pulomale

29. Electrocardiography (ECG)
RVH or RV strain
a. right axis deviation
b. R/S amplitude ratio in V1 greater than 1 R/S amplitude ratio in V6 less than 1
c. P-pulmonale -increase in P wave amplitude in leads 2, 3, and aVF

30. d. incomplete or complete right bundle branch block (RBB), especially if pulmonary embolism is the underlying etiology
e. low-voltage QRS because of underlying COPD with hyperinflation and increased AP diameter of the chest.

31. Chest roentgenography
enlargement of the central pulmonary arteries with oligemic peripheral lung fields- per. pruning
right descending pulmonary artery > 16 mm
left pulmonary artery >18 mm in diameter
R V H

34. Elevated brain natriuretic peptide (BNP) level
Earliest evidence of CCF
a natural mechanism to compensate for elevated pulmonary hypertension and right heart failure by
a. promoting diuresis and natriuresis,
b. vasodilating systemic and pulmonary vessels

35. Arterial blood gas tests
provide important information about the level of oxygenation and type of acid-base disorder

36. To know the etiology P F T to confirm underlying lung disease
To exclude pulmonary thromboembolism
Ventilation/perfusion (V/Q) scan or CT chest
Hypercoagulability states evaluated by levels of
proteins C and S
antithrombin III
factor V Leiden
antinuclear antibody (ANA) level for collagen vascular disease such as scleroderma
serum alpha1-antitrypsin

37. MANAGEMENT

38. Oxygen therapy
Diuretics
Vasodilators
Digitalis
Anticoagulation therapy
are all different modalities used in the long-term management of
Chronic cor pulmonale

39. long-term oxygen therapy can be considered even if
PaO2 is greater than 55 mm Hg or
O2 saturation is greater than 88%.
( because of vasodilator effect on pulmonary arteries)

40. DIURETICS Right ventricular filling volume markedly elevated
Diuretics may result in improvement of function of both the right and left ventricles
adverse effects.
a. Excessive volume depletion can lead to a decline in cardiac output
b. hypokalemic metabolic alkalosis lead to cardiac arrhythmia
Diuretics needs to be used with caution

41. Vasodilator drugs
In long-term management of chronic cor pulmonale have modest results
1.Calcium channel blockers
oral sustained-release nifedipine
diltiazem
2.beta blockers
3.Nitrates
4.angiotensin-converting enzyme (ACE) inhibitors
not routinely used. A trial of vasodilator therapy considered in patients with COPD with disproportionately high pulmonary blood pressure – more than 40 mm Hg

42. NEWER VASODILATORS endothelin receptor antagonist
(Bosentan)
prostacyclin PGI 2 analogues
Epoprostenol -i.v.
iloprost - M D I
THEY HAVE SHOWN A PROMISING EFFECT IN REDUCING THE PULMONARY HYPERTENSION

43. CARDIAC GLYCOSIDES NOT ROUTINELY INDICATED
Beneficial effect not as obvious as in LVF
modest effect of digitalis on failing right ventricle in chronic cor pulmonale
Must be used cautiously
should not be used during the acute phases of
respiratory insufficiency
Patients with hypoxemia or acidosis are at
increased risk of developing arrhythmias

44. Theophylline bronchodilatory effect
reduce pulmonary vascular resistance and pulmonary arterial pressures
weak inotropic effect and thus may improve right and left ventricular ejection
Strenghtens diaphragm
Stimulates the respiratory centre

45. 1. Reduction of blood viscosity
Phlebotomy
Mean Pul art press and PVR decrease in polycythemic patients after phlebotomy (hematocrit of >60 or 65)
The reduction of markedly elevated hematocrit level to about 50% by phlebotomy leads to
1. Reduction of blood viscosity
2. Reduction in PVR and pulmonary art pr
3. Improve gas exchange & increases exercise tolerance