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

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Presentation on theme: "HJS Constrictive Pericarditis Heiko J. Schmitt, M.D., Ph.D."— Presentation transcript:

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

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

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

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

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

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

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

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

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

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

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

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

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

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

15 HJS Constrictive Pericarditis - Clinic Braunwald, Heart Disease 4th ed., 1992 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

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

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

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

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

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

21 HJS u Useful in the differential diagnosis of constrictive pericarditis u Exclusion of other causes of right sided heart failure (valve disease, left sided heart failure, pulmonary hypertension). u 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: Constrictive Pericarditis - Echocardiography

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

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

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

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

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

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

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

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

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

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

34 HJS Constrictive pericarditis Cause-specific survival after pericardectomy J Am Coll Cardiol 2004;43: 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

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

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

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

38 Giessen, Germany

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