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constrictive pericarditis

Etiology: 1) most cases are idiopathic 2) post pericardiotomy syndrome following: a) cardiac surgery b) mediastinal irradiation 3) may occur as a late complication of pericarditis - tuberculous pericarditis 4) cardiac surgery [17] 5) connective tissue disorders 6) uremia [4] Pathology: 1) non compliant pericardium - pericardial thickening, fibrosis, calcification [4] 2) impairment of late diastolic cardiac ventricle filling - early diastolic cardiac ventricle filling unimpaired 3) reduced & fixed cardiac chamber volume 4) diminished cardiac output 5) decreased transmission of intrathoracic pressure results in enhanced ventricular interdependence [4] 6) elevation of central venous pressure - volume overload Clinical manifestations: 1) insidious onset a) fatigue b) exercise intolerance c) venous congestion 2) signs of right heart failure a) jugular venous distension (JVD) in > 90% - prominent x-descent & y-descent [4] - inspiratory elevation in JVD (Kussmaul's sign) b) peripheral edema c) hepatic congestion & hepatomegaly, ascites 3) pleural effusion [4] 4) pericardial knock during diastole difficult to distinguish from S3 5) absence of pulmonary congestion [4] - > 90% have clear lung on auscultation 6) diminished apical impulse 7) pulsus paradoxus in < 20% [4] Laboratory: - serum B-type natriuretic peptide (serum BNP) a) normal or minimally elevated; mean value 130 pg/mL [4] (> 800 pg/mL for restrictive cardiomyopathy) b) sensitive, but not specific test for distinguishing constrictive pericarditis from restrictive cardiomyopathy c) does NOT substitute for cardiac catheterization with hemodynamic assessment [4] - erythrocyte sedimentation rate may be elevated - complete blood count (CBC) may show leukocytosis - adenosine deaminase in pericardial fluid to rule out tuberculosis Special laboratory: 1) electrocardiogram - low voltage 2) echocardiography a) pericardial thickening b) respiratory variation in filling of right & left ventricles c) reduced diastolic filling with ventricular interdependence (diastolic filling of one chamber impedes diastolic filling of the other) manifested by 'to-&-fro' diastolic motion of the interventricular septum [4] d) exaggerated respiratory variation in mitral & tricuspid flow velocities e) ventricular septal shift during respiration f) may be pericardial effusion with effusive constrictive pericarditis - intrapericardial pressure reduced following drainage - intracardiac pressures unchanged following drainage g) right ventricular size & systolic function generally normal h) normal left ventricular size & function, no left ventricular hypertrophy i) myocardial thickness generally normal j) no right atrial & right ventricular diastolic collapse k) early mitral flow velocity increased l) plethora (dilation) of inferior vena cava [4] 3) cardiac catheterization if echocardiography indeterminate - demonstration of elevated & equalized diastolic pressures in all 4 cardiac chambers - elevated atrial pressures - LV diastolic pressure within 5 mm of Hg of RV diastolic pressure - prominent x-descent & y-descent Radiology: 1) chest X-ray may show pericardial calcification (generally absent) 2) chest CT or MRI of thorax - may show pericardial thickening, calcification - more sensitive than X-ray 3) radionuclide ventriculography - more rapid early diastolic filling in patients with constrictive pericarditis relative to restrictive cardiomyopathy Differential diagnosis: 1) restrictive cardiomyopathy* - pericardial knock during diastole absent in restrictive cardiomyopathy, but difficult to distinguish from S3 - restrictive cardiomyopathy more likely associated with amyloidosis (see Etiology: both disorders) - distinction is critical since surgery is not indicated for restrictive cardiomyopathy - doppler echocardiography & doppler velocity required to differentiate [4] 2) pericardial tamponade - early diastolic right ventricular collapse, hypotension & tachycardia consistent with pericardial tamponade 3) right ventricular myocardial infarction * see features distinguishing constrictive pericarditis Management: 1) high-dose NSAID or glucocorticoid for potentially transient constrictive pericarditis (see pericarditis) [1] - glucocorticoid + colchicine [4] 2) clinical improvement in 2-6 months (90%) 3) definitive therapy requires complete pericardiectomy a) perioperative mortality 5-10% b) indicated for patients with functional NYHA class II or class III heart failure [4] c) 2-3 months of anti-inflammatory therapy reasonable prior to pericardiectomy - MKSAP20 does not mention high perioperative mortality [4] - pericardial calcification on imaging & right heart failure are signs of chronic constrictive pericarditis & indications for pericardiectomy without a trial of anti-inflammatory therapy [4] 4) minimally symptomatic patients a) sodium & fluid restriction b) diuretics c) closely monitor for hemodynamic deterioration 5) patient education - hemodynamic & symptomatic relief in 2-6 months (90%) 6) follow-up: all patients to identify patients with extension to epicardium & myocardium

Related

features distinguishing constrictive pericarditis

Specific

effusive constrictive pericarditis

General

pericarditis

References

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  2. Saunders Manual of Medical Practice, Rakel (ed), WB Saunders, Philadelphia, 1996, pg 268-70
  3. Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 51-52
  4. Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2006, 2009, 2012, 2015, 2018, 2022. - Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022 - Medical Knowledge Self Assessment Program (MKSAP) 20 American College of Physicians, Philadelphia 2025
  5. Harrison's Principles of Internal Medicine, 13th ed. Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 1097
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  17. NEJM Knowledge+
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