1. CorPulmonale Sung Chul Hwang, M.D. Dept. of Pulmonary and Critical Care Medicine Ajou University School of Medicine

2. Cor Pulmonale • Right Sided Heart Disease,secondarily caused by abnormalities of lung parenchyme, airways, thorax, or respiratory control mechanisms. • Noevidence of other heart conditions, • Acute vs. Chronic

3. Lung and Airways COPD Asthma Bronchiectasis DILD Pulmonary tuberculosis Vascular Occlusion Multiple Emboli Schistosomiasis Filariasis Sickle Cell P. Pulmonary Hypertension Etiology of Cor Pulmonale ( I )

4. Thoracic Cage Kyphosis > 100 o Scoliosis > 120 o Thoracoplasty Pleural fibrosis N-M Disease Polio Myelitis Myasthenia Gravis ALS Muscular Dystrophy Etiology of Cor Pulmonale ( II )

5. Etiology of Cor Pulmonale ( III ) Abnormal Respiratory Control • Idiopathic hypoventilation Syndrome • Obesity hypoventilation syndrome (Pick-Wickian syndrome) • Cerebrovascular disease

6. Hypercapnea H Anatomic changes Hypoxia Acidemia A Pulmonary Vessel Restriction Increased Viscosity Increased C.O. C Acidosis Chronic Cor Pulmonale Rt. Ventricular Failure

7. Pathologic Features • Lung : consistent with Specific diseases • Common Features: hypertrophy of microvasculatures • Hallmark : Rt. Ventricular Hypertrophy 60g – 200g, > 0.5 CM, RV/LV <2.5 • Lt. Ventricular Hypertrophy • Hypertrophy of Carotid Body

8. Natural History • Several months to years to develop • All ages from child to old people • Repeated infections aggravate RV strain into RV failure • Initilly respondes well to therapy but progressively becomes refractory

9. Prevalence • Emphysema : less frequent • Cronic bronchitis : more common • US : 6-7 % of Heart failure • Delhi : 16% • Sheffield in UK : 30 – 40% • Autopsy in Chronic Bronchitis : 50% • More prevalent in pollution area or smokers

10. Lab. Findings • X-Ray : Prominent pulmonary hilum pulmonary artery dilatation Rt MPA > 20 mm • EKG : P- pulmonale, RAD, RVH • Echocardiography : RVH, TR, Pulm. Hypertension • ABG : Hypoxemia, Hypercapnea, Respiratory acidosis • CBC : polycythemia • Cardiac catheterization

11. Treatment • Treat Underlying Disease : COPD Tx, Steroid, Infection control, theophylline, medroxyprogesterone, • Continuous O2 : < 2-3L/min • Diuretics • Phlebotomy • Digoxin : controversial • Pul. Vasodilators • Beta adrenergic agents • Reduce Ventilation/Perfusion imbalance : Amitrine bimesylate

12. Prognosis • 1960-1970 : 3 yr mortality 50-60% • Recent times : 5 - 10 years or more