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HJS
Constrictive Pericarditis
Heiko J. Schmitt, M.D., Ph.D.
HJSOutline
Case presentation Pericardial anatomy Clinical presentation and exam CT, MRI, and echocardiographic findings Hemodynamics Outcome after pericardectomy
HJS
NEJM 2004, Vol 351, 1014-9
Case Presentation - History
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
HJS
NEJM 2004, Vol 351, 1014-9
Case Presentation - Exam
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
HJS
NEJM 2004, Vol 351, 1014-9
Case Presentation - Initial Tests
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
HJS
NEJM 2004, Vol 351, 1014-9
Case Presentation - Initial Tests
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 diureticsWorsening of symptoms
HJS
NEJM 2004, Vol 351, 1014-9
Case Presentation - Final Tests
Mild cardiomegaly increased interstitial markings
No pulmonary disease but thickened pericardium
HJS
NEJM 2004, Vol 351, 1014-9
Case Presentation - Heart Catheter
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.
HJSPericardium - Anatomy
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.
HJSPericardium - Anatomy
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
HJSPericardium - Anatomy
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.
HJSConstrictive Pericarditis - Etiology
Who develops constriction?
Fibrinous Hemorrhagic Purulent
HJSConstrictive Pericarditis - Etiology
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
HJSConstrictive Pericarditis - Pathophysiology
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
HJSConstrictive 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
HJSConstrictive Pericarditis - Exam
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
HJSConstrictive Pericarditis - Exam
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
HJSConstrictive Pericarditis - Exam
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
HJSConstrictive Pericarditis - CXR
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
HJSConstrictive Pericarditis - CT/MRI
May show thickened pericardium
May exclude other abnormalities.
Normal pericardium however does not exclude restrictive pericarditis.
Nishimura, Heart 2001, 86, 619-23
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 - Echocardiography
HJSConstrictive Pericarditis - 2D Echo
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.
HJSConstrictive Pericarditis - Doppler
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
HJSConstrictive Pericarditis - Doppler
Corresponds with right atrial filling Prominent a-wave deep y-descent
Otto, Textbook of clinical Echocardiography, 3rd ed.
a
xv
y
High initial E velocity short deceleration time reduced velocity at atrial contraction Decrease in E velocity during inspiration
HJSConstrictive Pericarditis - Echocardiography
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
HJSConstrictive Pericarditis - Catheterization
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
HJSConstrictive Pericarditis - Catheterization
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
Grossman Cardiac catheterization, Angiography, and Intervention, 2000 6th edition
a v
HJSConstrictive Pericarditis - Catheterization
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
HJSConstrictive Pericarditis - Catheterization
Increase of RA pressure during inspiration
Kussmaul’s sign
Grossman Cardiac catheterization, Angiography, and Intervention, 2000 6th edition
HJSConstrictive Pericarditis - Restrictive CM
Otto, Textbook of clinical Echocardiography, 3rd ed.
HJSConstrictive Pericarditis - Restrictive CM
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
HJSConstrictive Pericarditis - Mortality
Etiology NYHA III-IV marked elevation of RV
end-diastolic pressure
Braunwald, Heart Disease 4th ed., 1992
11%
15%
5%
Perioperative Mortality
1980
1990
2004
HJS
Constrictive pericarditis Cause-specific survival after pericardectomy
J Am Coll Cardiol 2004;43:1445-52
Pericardectomy at the Cleveland clinic foundation January1977-December 2000, 163 patients
Idiopathic 75 (46%)
Postsurgical 60 (37%)
Irradiation 15 (9%)
Miscellaneous 13 (8%)
Perioperative MortalityLong term Survival
HJS
Constrictive pericarditis Cause-specific survival after pericardectomy
J Am Coll Cardiol 2004;43:1445-52
Overall perioperative mortality 6.1%
Idiopathic 2.7%
Postsurgical 8.3%
Irradiation 21.4%
Miscellaneous 0%
HJS
Constrictive pericarditis Cause-specific survival after pericardectomy
J Am Coll Cardiol 2004;43:1445-52
Idiopathic 88% 7-year survival
postsurgical 66% 7-year survival
irradiation 27% 7-year survival
HJSConstrictive Pericarditis - Summary
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
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