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

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

3. Case Presentation - History
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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,

4. 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,

5. Case Presentation - Initial Tests
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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,

6. Case Presentation - Initial Tests
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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,

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

8. Case Presentation - Heart Catheter
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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,

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.

10. Pericardium - Anatomy Outer fibrous layer
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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

11. Pericardium - Anatomy
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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.

12. Constrictive Pericarditis - Etiology
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Purulent
Fibrinous
Hemorrhagic
Who develops constriction?

13. Constrictive Pericarditis - Etiology
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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

14. Constrictive Pericarditis - Pathophysiology
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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

15. Constrictive Pericarditis - Clinic
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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

16. Constrictive Pericarditis - Exam
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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,

17. 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,

18. Constrictive Pericarditis - Exam
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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

19. Constrictive Pericarditis - CXR
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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

20. Constrictive Pericarditis - CT/MRI
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May show thickened pericardium
May exclude other abnormalities.
Normal pericardium however does not exclude restrictive pericarditis.
Nishimura, Heart 2001, 86,

21. Constrictive Pericarditis - Echocardiography
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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

22. Constrictive Pericarditis - 2D Echo
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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.

24. Constrictive Pericarditis - Doppler
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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

25. 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.

26. Constrictive Pericarditis - Echocardiography
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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

27. Constrictive Pericarditis - Catheterization
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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, th edition

28. 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, th edition

29. Constrictive Pericarditis - Catheterization
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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

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

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

32. Constrictive Pericarditis - Restrictive CM
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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, th edition

33. Constrictive Pericarditis - Mortality
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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

34. 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:

35. Constrictive pericarditis Cause-specific survival after pericardectomy
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Overall perioperative mortality 6.1%
Idiopathic 2.7%
Postsurgical 8.3%
Irradiation 21.4%
Miscellaneous 0%
J Am Coll Cardiol 2004;43:

36. Constrictive pericarditis Cause-specific survival after pericardectomy
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Idiopathic 88% 7-year survival
postsurgical 66% 7-year survival
irradiation 27% 7-year survival
J Am Coll Cardiol 2004;43:

37. Constrictive Pericarditis - Summary
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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.

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