Cor Pulmonale, also known as pulmonary heart disease, is caused by high blood pressure in the lungs secondary to diseases affecting the lungs, chest wall, or pulmonary vasculature. It is characterized by hypertrophy and dilation of the right ventricle of the heart in response to the elevated pulmonary pressures. The main symptoms include cough, sputum production, shortness of breath, fatigue, and leg swelling. Diagnosis involves assessing the patient's medical history along with tests such as chest x-rays, electrocardiograms, echocardiograms, and blood gas analysis. Treatment focuses on controlling infections, improving oxygen levels, managing heart failure, and preventing complications through patient monitoring and education.

1. Cor Pulmonale (Pulmonary Heart Disease) Guo Yubiao, M.D & Ph.D Pulmonary & Critical Care Medicine The first Affiliated Hospital of Sun-Yat Set University

3. Presentation Outline Definition Epidemiology/Classification Clinical manifestations Symptoms Signs Diagnostic workup & Differential Diagnosis Diagnosis Differential Diagnosis Treatment & Prevention Summary

4. Classification Acute Cor Pulmonale Massive pulmonary embolism Chronic Cor Pulmonale

5. Epidemiology High prevalence(1992, 4.42 ‰) District difference Smoking Acute exacerbation

6. Definition of Chronic Cor Pulmonale Hypertrophy and dilatation of the right ventricle Secondary to the pulmonary hypertension Caused by disease of the pulmonary parenchyma, and/or chest wall, and/or pulmonary vascular system With or without right heart failure Exclusion the causes of congenital heart disease and left heart disease

7. Etiology Pulmonary parenchyma disease COPD80%~90%,asthma,bronchiectasis,severe tuberculosis, idiopathic interstitial pneumonia, sarcoidosis, eosinophilic granuloma,silicosis, etc . Disorders of the neuromuscular apparatus and chest wall Poliomyelitis ,Guillain-Barr é syndrome, Kyphoscoliosis

8. Etiology Pulmonary vascular disorders chronic pulmonary thromboembolism, pulmonary arteriolitis, allergic granulomatosis, primary pulmonary hypertension Others primary alveolar hypoventilation, sleep apnea syndromes

9. Pathophysiology The formation of pulmonary hypertension Functional factors of increase of vascular resistance Anatomical factors of increase of vascular resistance Increased blood volume and hyperviscosity Cardiac disorders and heart failure Damage to other vital organs

10. Functional factors of increase of vascular resistance Hypoxia is the most important factor for pulmonary hypertention. Hypoxic pulmonary vasoconstriction Determined mostly by the ratio of vasoconstrictive substances to vasodilative substances Leukotriene, 5-HT, Ang II, PAF,EDCF/ NO, PGI 2 ,EDRF Direct effect of hypoxia on the increase of the smooth muscular cell membrane permeability to Ca 2+ Acidosis increases the sensitivity of vasoconstriction to hypoxia

11. Anatomical factors of increase of vascular resistance Vasculitis Emphysema, increased intra-alveolar pressure, compressed pulmonary capillaries Reduction in pulmonary capillary bed Pulmonary vascular remodeling Multiple pulmonary micro-arteriole thrombosis

12. Increased blood volume and hyperviscosity Secondary polycythemia and hyperviscosity Water and sodium retention Aldosterone Renal arteriole constriction

13. Cardiac disorders and heart failure Right ventricular hypertrophy secondary to pulmonary hypertension. Sustained pulmonary hypertension exceeds the compensation of right ventricle, and causes the increase of right ventricular end diastolic pressure, and dilation and failure of right ventricle. A few may develop left heart failure.

14. Damages to other vital organs Hypoxia and acidosis can also do damages to other vital organs, e.g. brain,liver,kidney,gastrointestine, endocrine system .

15. Pulmonary diseases Hypoxia, hypercapnia/acidosis Pulmonary Destruction of capillary bed Blood volume ↑ vasoconstrition Blood viscosity ↑ Pulmonary hypertension Right heart workload ↑ Right ventricular hypertrophy Right heart failure Impaired Toxic effect from bacteria Cardiac myocardial function on myocardium arrythmia Myocardial hypoxia Recurrent Electrolytic and Accumulation of lactate pneumonia acid-base disturbance

16. Clinical manifestations Compensatory stage of the respiratory and cardiac function De-compensatory stage of the respiratory and cardiac function

17. Compensatory stage of the respiratory and cardiac function Symptoms: cough, sputum, short of breath, dyspnea and palpitation on exertion, fatigue and decrease of exercise tolerance more severe in acute exacerbation. A few with chest pain or hemoptysis .

18. Compensatory stage of the respiratory and cardiac function Signs: cyanosis, signs of emphysema, moist rales and/or rhonchi, distal heart sound, systolic murmur of tricuspid area , P2>A2 , subxiphoid visible/palpable cardiac impulse, distended jugular venous pulsation

19. De-compensatory stage of the respiratory and cardiac function Respiratory failure Symtoms : severe dyspnea,especially at night, headache, insomnia,inappetence, somnolence, dizziness,confusion, even delirium. Signs : congunctiva congestion and edema, retinal vasodilatation, optic papillary edema. weakness or disappear of deep reflexes, pathological reflexes, redness, sweaty

20. Right heart failure Symptoms: predominant short of breath,palpitation, inappetence,nausea Signs: cyanosis,tachycardia,arrhythmia,subxiphoid systolic murmur or even diastolic murmur. Tender hepatomegaly,Hepatojugular reflux, lower extremity edema, ascites

21. signs

22. Chest radiography Signs of pulmonary hypertension: Enlarged right descending pulmonary artery with diameter ≥15mm The ratio of the diameter of right descending pulmonary artery to trachea≥1.07 Bulge of pulmonary artery/with the height≥3mm dilation of the main pulmonary artery and its branches with concurrent underperfusion of the peripheral branches Signs of right ventricular enlargement Signs of underlying diseases and infection

24. Electrocardiography Right-axis deviation with a frontal plane axis greater than +90 ° Marked clockwise rotation of the electrical axis R v1 +S v5 ≥ 1.05mv P-pulmonale pattern Incomplete or rarely complete right bundle branch block Low voltage QRS Occasional large Q wave or QS in V1,V2, even V3,suggesting healed myocardial infarction

25. Electrocardiography of Chronic Cor Pulmonale

26. Echocardiography The right ventricular outflow ≥ 30 m m The right ventricular internal dimension ≥20mm Anterior RV wall thickness Ratio of left to right ventricular internal dimension <2 Increased right pulmonary artery or pulmonary artery dimension (Peak systolic pulmonary artery pressure) Increased right atrium dimension

27. Arterial blood gas analysis Hypoxemia and/or hypercapnia Respiratory failure: PaO2<60mmHg PaCO2>50mmHg

28. Serum assessment Increased RBC count and Hemoglobin Increased blood and plasma viscosity Increased WBC count and neutrophilic ratio when infection occurs Change of renal or hepatic function Electrolyte imbalance

29. Others Pulmonary function test For early stage or non-exacerbation stage patients Sputum culture For guidance of antibiotics selection in patients with acute exacerbation stage

30. Diagnosis History of COPD and pulmonary parenchymal or chest wall or pulmonary vascular disease, Symptoms and signs of pulmonary hypertension and right heart dilation or failure, combined with the signs of right heart hypertrophy and dilation by ECG, X-ray,UCG.

31. Differential Diagnosis Coronary atherosclerotic heart diseases History of left heart failure, hypertension, hyperlipoidemia ； Symtoms of angina pectoris, myocardial infarction Left ventricular hypertrophy in P.E. and X-ray, myocardial ischemia in ECG Rheumatic heart diseases History of rheumatic arthritis and myocarditis Usu. involving other cardiac valves Special signs in X-ray, ECG and UCG Primary cardiomyopathy No history of chronic pulmonary diseases Enlargement of entire heart No signs of pulmonary hypertension in X-ray

32. Therapy S tage of Acute exacerbation Control of infection Oxygen therapy Control of heart failure Control of cardiac arrythmia Anticoagulatory therapy Patient care

33. Control of infection Antibiotics based on sputum culture Before the culture, based on infection acquired location and sputum smear gram stain CAP: G positive; HAP:G negative Penicillins, aminoglycerides,quinolones and cephalosporins Secondary fungal infection

34. Oxygen therapy Clearance of respiratory tract Correction of hypoxia and hypercapnia

35. Control of heart failure Different to heart failure caused by other cardiac disease May be improved after control of infection and normality of blood gas Diuretics Vasodilators Positive inotropic agents-digitalis

36. Diuretics Reduction of blood volume and right heart load,elimination of edema Small dosage and short period Moderate degree:HCT .antisterone;Severe cases: furosemide Adverse effect: alkalosis with low K + &CL - thickened sputum blood condensation

37. Vasodilators Reduction of cardiac pre- and after- load and oxygen consumption, improve the myocardial contractibility. Adverse effect: low blood pressure, tachycardia and hypoxemia and hypercapnia

38. Positive inotropic agents-digitalis Low tolerance to digitalis due to chronic hypoxia and infection: arrhythmia Correction of hypoxia and hypokalemia before use Low dosage, fast action and fast metabolite agents: lanatoside C(cedilanid),strophanthin K Indications: refractory edema after improvement of infection and respiratory function and no effect on diuretics right heart failure without obvious infection acute left heart failure

39. Control of arrhythmia:usually self-limited Anticoagulatory therapy:heparin or low molecular weight heparin Intensive patient care:monitoring, airway secretion aspiration

40. Stage of non-exacerbation Combined with Chinese medicine Long-term home oxygen therapy Pulmonary Rehabilitation Regulation of immune system Nutrition

41. Complications Pulmonary encephalopathy Acid-base and electrolyte disturbance Cardiac arrhythmia:af,aF,at Shock Gastrointestional haemorrhage Disseminated intravascular coagulation

42. Prognosis Recurrent acute exacerbation, progressing with gradually impairment of pulmonary function Motality of 10%-15% Prolong lifespan and improve quality of life

43. Prevention Smoking cessation Treatment of the predisposing factors respiratory tract infection, inhalation of noxious gases, occupational protection, etc Education

44. Thank You!