Diagnosis and management.

1. Cor Pulmonale
Presented By
Dr. Md. Shafiqul Islam Dewan
Resident (Pulmonology) - Phase-A
Dhaka Medical College Hospital

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.

4. Classification
1. Acute Cor Pulmonale
2. Chronic Cor pulmonale

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.

8. Etiology of Acute Cor Pulmonale
 massive pulmonary embolism
 acute respiratory distress syndrome (ARDS)

9. Etiology of Chronic Cor Pulmonale
 Diseases of the Lung Parenchyma
 Chronic obstructive pulmonary disease
 Emphysema
 Chronic bronchitis
 Cystic fibrosis
 Idiopathic interstitial pneumonitis
 Idiopathic pulmonary fibrosis
 Nonspecific interstitial pneumonitis
 Sarcoidosis
 Bronchiectasis
 Pulmonary Langerhans cell histiocytosis
 Lymphangioleiomyomatosis

10. Etiology of Chronic Cor Pulmonale
 Diseases of the Pulmonary Vasculature
 Pulmonary arterial hypertension (PAH)
Idiopathic PAH
Heritable PAH
Associated PAH
 Venoocclusive disease
 Chronic thromboembolic pulmonary hypertension
 Pulmonary tumor thrombotic microangiopathy

11. Etiology of Chronic Cor Pulmonale
 Disorders of Chronic (Alveolar) Hypoxia
 Alveolar hypoventilation syndromes
 Obesity hypoventilation syndrome
 Central hypoventilation syndrome
 Neuromuscular respiratory failure
 Chest wall disorders
Kyphoscoliosis
 Living at high altitude

12. Clinical Presentation
 Symptoms
 Dyspnea
 Cough
 Lower-extremity edema
 Abdominal pain and ascites
 Rarely Orthopnea and PND
 nonspecific symptoms
 Fatigue,
 palpitations,
 atypical chest pain,
 dizziness,
 syncope

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.

17. Management
 General management
 Specific management
 Management of complication
 Management of acute exacerbation

18. General management
 Education and reassurance
 Oxygen therapy
 Low-level graded aerobic exercise
 Avoid heavy physical exertion
 Sodium-restricted diet (<2400 mg/d)
 Routine immunization
 Smoking cessation

19. Specific management
 Treatment of underlying cause.
 Treatment of pulmonary hypertension.

20. Treatment of pulmonary hypertension
 Endothelin receptor antagonists
 Ambrisentan
 Bosentan
 Macitentan
 Prostacyclin (PGI2) analogues
 Epoprostenol - intravenous formulation
 Treprostinil - subcutaneous injection
 Iloprost – inhaled formulation
 Phosphodiesterase type 5 (PDE5) inhibitors
 Sildenafil
 Tadalafil
 Vardenafil
 Calcium channel blockers
 Diltiazem

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.