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

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

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

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

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

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

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

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

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.

Pericardium - Anatomy Outer fibrous layer 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

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.

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

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

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

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

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

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

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

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

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

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

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.

Constrictive Pericarditis - Doppler HJS Doppler echocardiography provides useful information in patients with constrictive pericarditis. 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

Constrictive Pericarditis - Doppler HJS a v x y Corresponds with right atrial filling Prominent a-wave deep y-descent 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.

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

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

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 a v Grossman Cardiac catheterization, Angiography, and Intervention, 2000 6th edition

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

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

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

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

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

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

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

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

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.

Giessen, Germany

The Kids