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Cor pulmonale - october'18
Cor pulmonale is right ventricular heart failure caused by lung disease that leads to pulmonary hypertension. The most common cause is chronic obstructive pulmonary disease. It can be acute or chronic. Treatment focuses on managing the underlying lung condition, treating pulmonary hypertension with medications, diuretics for volume overload, anticoagulation for those at high risk of clots, and oxygen therapy. Prognosis depends on the specific lung disease, with cor pulmonale due to conditions like COPD generally having a poorer long term outlook.
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Cor pulmonale - october'18
- 1. Cor Pulmonale Presented By Dr.Md. Shafiqul Islam Dewan Resident (Pulmonology) - Phase-A Dhaka Medical College Hospital
- 2. Definition Cor pulmonaleis 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 pulmonaleis 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 CorPulmonale 2. Chronic Cor pulmonale
- 5. Pathophysiology Common pathophysiologicmechanism 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 responseof 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 consequencesof 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 AcuteCor Pulmonale massive pulmonary embolism acute respiratory distress syndrome (ARDS)
- 9. Etiology of ChronicCor 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 ChronicCor 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 ChronicCor 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 seeright ventricular dysfunction and pulmonary hypertension ECG Echocardiography Right heart catheterization Cardiac magnetic resonance
- 15. Investigation To identifythe 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 Educationand 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 Treatmentof underlying cause. Treatment of pulmonary hypertension.
- 20. Treatment of pulmonaryhypertension 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 Phlebotomyis indicated in patients with chronic cor pulmonale and chronic hypoxia causing severe polycythemia Single-lung, double-lung, and heart-lung transplantation
- 23. Management of acuteexacerbation Hospitalization Oxygen therapy Control of heart failure Control of cardiac arrhythmia Control of infection Treatment of lung disease accordingly
- 24. Prognosis The prognosisof cor pulmonale is variable depending upon the underlying pathology. Development of cor pulmonale as a result of a primary pulmonary disease usually poor prognosis.