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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) jugular venous distension (JVD) in > 90%
a) prominent x-descent & y-descent [4]
b) inspiratory elevation in JVD (Kussmaul's sign)
3) peripheral edema
4) hepatic congestion & hepatomegaly, ascites
5) pleural effusion [4]
6) pericardial knock during diastole difficult to distinguish from S3
7) absence of pulmonary congestion [4]
- > 90% have clear lung on auscultation
8) diminished apical impulse
9) 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
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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McKenzie (eds), Little, Brown & Co, Boston, 1995, pg 135
- Saunders Manual of Medical Practice, Rakel (ed), WB Saunders,
Philadelphia, 1996, pg 268-70
- Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed)
Lippincott-Raven, Philadelphia, 1998, pg 51-52
- 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
- Harrison's Principles of Internal Medicine, 13th ed.
Isselbacher et al (eds), McGraw-Hill Inc. NY, 1994, pg 1097
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- NEJM Knowledge+