1. Constrictive Pericarditis Heiko J. Schmitt, M.D., Ph.D. HJS

2. Outline HJS • Case presentation • Pericardial anatomy • Clinical presentation and exam • CT, MRI, and echocardiographic findings • Hemodynamics • Outcome after pericardectomy

3. Case Presentation - History HJS • 67 year old man presents with a 2 months history of SOB, non-productive cough and b/l swelling of his lower extremity. • occasional wheezing and more SOB after meals • symptoms started after a hunting trip • no constitutional symptoms • no lung disease or heart disease, occupational exposure, allergies, smoking history • History is remarkable for GERD and a remote pneumonia NEJM 2004, Vol 351, 1014-9

4. Case Presentation - Exam HJS • Because of worsening symptoms admission • Patient now reported orthopnea • afibrile, BP 150/86, HR 108, RR 28 • expiratory wheezes over both lungs • no M/R/G, distant heart sounds • 2+ pitting leg edema b/l • JVP not visualized • His weight is 109 kg NEJM 2004, Vol 351, 1014-9

5. Case Presentation - Initial Tests HJS • Labs were unremarkable including CBC, BMP, CPK, Troponin, LFTs • ph 7.47, pCO2 34, pO2 64 • CXR: Cardiomegaly and mildly increased vasculature • EKG: showed diffuse T-wave inversion, low voltage and sinustachycardia • Echo: nl LV size and function, RV nl. size but thickened, no valvular disease • Dobutamin-stress: no evidence for ischemia NEJM 2004, Vol 351, 1014-9

6. Case Presentation - Initial Tests HJS • Spiral-CT: no evidence for PE, right sided pleural effusion, no infiltrate • PFTs: FVC 2.5l (59%), FEV1 1.9l (65%), ratio 76%, TL 5.4l (85%). • Sleep-Study: 21 apneic, 12 hypopneic episodes per hour, desaturation to 83%. Started on nocturnal CPAP and diuretics Worsening of symptoms NEJM 2004, Vol 351, 1014-9

7. Case Presentation - Final Tests HJS • Mild cardiomegaly • increased interstitial markings • No pulmonary disease but thickened pericardium NEJM 2004, Vol 351, 1014-9

8. Case Presentation - Heart Catheter HJS • Hemodynamic measurements were consistent with the diagnosis of constrictive pericarditis • Elevated and equal enddiastolic pressures • Discordant peak sytolic pressures • The patient underwent pericardectomy showing fibrosed pericardium and did well. NEJM 2004, Vol 351, 1014-9

9. Pericardium - Anatomy HJS • Forms a sac enclosing the origin of the aorta, pulmonary artery, Pulmonary veins, venae cavae • ligamentous attachments to sternum, vertebral column, and diaphragm • ligaments help to fix the heart anatomically and prevent excessive movements Otto, Textbook of clinical Echocardiography, 3rd ed.

10. Pericardium - Anatomy HJS • Outer fibrous layer • Inner parietal layer forming a serous membrane composed of a single layer of mesothelial cells • Visceral layer is firmly attached to the surface of the heart

11. Pericardium - Anatomy HJS • Marked increase in surface area of the visceral pericardium by microvili and cilia. • Microvilli and cilia permit movement and fluid transport • Pericardial fluid is an ultrafiltrate of plasma (nl 50ml) • contains phospholipids that serve as a lubricant.

12. Constrictive Pericarditis - Etiology HJS Purulent Hemorrhagic Fibrinous Who develops constriction?

13. Constrictive Pericarditis - Etiology HJS • Idiopathic 42% (earlier inapparent viral pericarditis) • Cardiac surgery 29% • Radiation therapy to the mediastinum • Renal failure • Connective tissue disease • TB (still highest in developing countries) • less common in children (suspect TB) Braunwald, Heart Disease 4th ed., 1992

14. Constrictive Pericarditis - Pathophysiology HJS • Fibrosed or calcified pericardium restricts diastolic filling of all 4 chambers • constriction leads to elevated and equilibrium of the diastolic pressures • In early diastole filling is unimpaired => abnormally rapid filling • filling is abruptly halted when cardiac volume meets the limits determined by the stiff pericardium • Virtually all filling occurs during early diastole Braunwald, Heart Disease 4th ed., 1992

15. Constrictive Pericarditis - Clinic HJS Systemic venous congestion Elevated left filling pressure Decreased cardiac output • Edema • Abdominal • swelling and • discomfort 2nd to • ascites • fullness, anorexia • exertional dyspnea • cough • orthopnea • fatique • muscle wasting • poor exercise • tolerance Braunwald, Heart Disease 4th ed., 1992

16. Constrictive Pericarditis - Exam HJS • Kussmaul’s sign (increase of RA pressure during inspiration). • described 1873 in combination with pulsus paradoxus in a patient with constrictive pericarditis. • In Mayo clinic series found in 21% of patients referred for pericardectomy. • Pulsus paradoxus (decrease in systolic pressure > 10 mmHg) infrequently found in constrictive pericarditis Lancet 2002; 359, 1940-42

17. Constrictive Pericarditis - Exam HJS • Kussmaul’s sign (increase of RA pressure during inspiration). • described 1873 in combination with pulsus paradoxus in a patient with constrictive pericarditis. • In Mayo clinic series found in 21% of patients referred for pericardectomy. • Pulsus paradoxus (decrease in systolic pressure > 10 mmHg with inspiration) found in 20% in constrictive pericarditis Lancet 2002; 359, 1940-42

18. Constrictive Pericarditis - Exam HJS • Pericardial knock heard over the left sternal border. • Corresponds with the sudden cessation of ventricular filling. • Earlier than S3 and higher frequency • may be confused with opening sound of mitral stenosis. Braunwald, Heart Disease 4th ed., 1992

19. Constrictive Pericarditis - CXR HJS • Normal heart 33% • Enlarged heart 67% • Pericardial calcification 43% • Pleural effusion 83% • Pulmonary venous congestion 86% • Left atrial enlargement 85% • Right superior mediastinum might be enlarged (sup. vena cava). Braunwald, Heart Disease 4th ed., 1992 Pulvaneswary: Constrictive Pericarditis, Australas.Radiol. 26:53, 1982

20. Constrictive Pericarditis - CT/MRI HJS • May show thickened pericardium • May exclude other abnormalities. • Normal pericardium however does not exclude restrictive pericarditis. Nishimura, Heart 2001, 86, 619-23

21. Constrictive Pericarditis - Echocardiography HJS • Useful in the differential diagnosis of constrictive pericarditis • Exclusion of other causes of right sided heart failure (valve disease, left sided heart failure, pulmonary hypertension). • Thickened ventricular walls with unusual texture found in restrictive and infiltrative CM are usually not found in restrictive pericarditis Nishimura R., Contrictive pericarditis in the modern era: a diagnostic dilemma, heart 2001;86:619-23

22. Constrictive Pericarditis - 2D Echo HJS • Pericardial thickening. • abrupt posterior motion of the ventr. septum in early diastole • abrupt anterior motion following atrial contraction • inspiratory septal shift • dilated inf. vena cava Otto, Textbook of clinical Echocardiography, 3rd ed.

24. Constrictive Pericarditis - Doppler HJS • Doppler echocardiography provides useful information in patients with constrictivepericarditis. • The pathophysiologic features of constrictive pericarditis (diastolic filling) are assessed by the analysis of • the mitral inflow • hepatic vein flow • pulmonary vein flow • Similar flow pattern can be found in restrictive cardiomyopathy

25. Constrictive Pericarditis - Doppler HJS • Corresponds with right atrial filling • Prominent a-wave • deep y-descent a v x y • High initial E velocity • short deceleration time • reduced velocity at atrial contraction • Decrease in E velocity during inspiration Otto, Textbook of clinical Echocardiography, 3rd ed.

26. Constrictive Pericarditis - Echocardiography HJS • A comprehensive echocardiogram may be considered diagnostic in a subset of patients with classical findings • septal bounce • respiratory septal shift • typical doppler findings with respiratory variation • pericardial thickening • However in up to 1/3 of the patients the echocardiographic findings are equivocal • combination of pericardial and myocardial disease • COPD • AFIB Nishimura R., Contrictive pericarditis in the modern era: a diagnostic dilemma, heart 2001;86:619-23

27. Constrictive Pericarditis - Catheterization HJS • Confirm presence of restrictive physiology and assess severity • differentiating constrictive pericarditis from restrictive cardiomyopathy • exclude major coexisting caused such as severe pulmonary hypertension • exclude rare causes of valvular constriction or pinching of coronary arteries. Grossman Cardiac catheterization, Angiography, and Intervention, 2000 6th edition

28. Constrictive Pericarditis - Catheterization HJS • Elevated RA pressure • very prominent Y decent indicating rapid RA emtying • Nadir of Y descent corresponds to the abrupt cessation of early diastolic ventricular filling • Characteristic W or M form v a Grossman Cardiac catheterization, Angiography, and Intervention, 2000 6th edition

29. Constrictive Pericarditis - Catheterization HJS • Left and right ventricular pressures should be recorded simultaneously at the same scale • RV and LV diastolic pressures are elevated and equal within 5 mm or less • dip and plateau configuration of RV and LV wave forms • all filling occurs during early diastole • tachycardia may obscure some of the findings Braunwald, Heart Disease 4th ed., 1992

30. Constrictive Pericarditis - Catheterization HJS • Increase of RA pressure during inspiration • Kussmaul’s sign Grossman Cardiac catheterization, Angiography, and Intervention, 2000 6th edition

31. Constrictive Pericarditis - Restrictive CM HJS Otto, Textbook of clinical Echocardiography, 3rd ed.

32. Constrictive Pericarditis - Restrictive CM HJS • Ventricular interdependence not seen in restrictive cardiomyopathy • Discordant change in left and right peak systolic pressure with repiratory changes. Grossman Cardiac catheterization, Angiography, and Intervention, 2000 6th edition

33. Constrictive Pericarditis - Mortality HJS • Etiology • NYHA III-IV • marked elevation of RV end-diastolic pressure Perioperative Mortality 15% 1980 11% 1990 5% 2004 Braunwald, Heart Disease 4th ed., 1992

34. Constrictive pericarditis Cause-specific survival after pericardectomy HJS Pericardectomy at the Cleveland clinic foundation January1977-December 2000, 163 patients Idiopathic 75 (46%) Postsurgical 60 (37%) Irradiation 15 (9%) Miscellaneous 13 (8%) Perioperative Mortality Long term Survival J Am Coll Cardiol 2004;43:1445-52

35. Constrictive pericarditis Cause-specific survival after pericardectomy HJS Overall perioperative mortality 6.1% Idiopathic 2.7% Postsurgical 8.3% Irradiation 21.4% Miscellaneous 0% J Am Coll Cardiol 2004;43:1445-52

36. Constrictive pericarditis Cause-specific survival after pericardectomy HJS • Idiopathic 88% 7-year survival • postsurgical 66% 7-year survival • irradiation 27% 7-year survival J Am Coll Cardiol 2004;43:1445-52

37. Constrictive Pericarditis - Summary HJS • Contrictive Pericarditis is a rare disease often posing a diagnostic challenge. • Echocardiography is an essential part in the diagnostic process and the diagnosis can be made if the classical fechocardiographic features are present. • Outcome after pericardectomy is excellent except in patients with irradiation as cause.

38. Giessen, Germany

39. The Kids