Presentation on theme: "HJS Constrictive Pericarditis Heiko J. Schmitt, M.D., Ph.D."— Presentation transcript:
HJS Constrictive Pericarditis Heiko J. Schmitt, M.D., Ph.D.
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
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
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
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
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
HJS NEJM 2004, Vol 351, Case Presentation - Final Tests u Mild cardiomegaly u increased interstitial markings u No pulmonary disease but thickened pericardium
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.
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.
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
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.
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
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
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,
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,
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
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
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,
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
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.
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
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
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
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
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
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
HJS Constrictive Pericarditis - Catheterization u Increase of RA pressure during inspiration u Kussmaul’s sign Grossman Cardiac catheterization, Angiography, and Intervention, th edition
HJS Constrictive Pericarditis - Restrictive CM Otto, Textbook of clinical Echocardiography, 3rd ed.
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
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
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
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
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.