Transcript Pericardial Constriction

Constrictive Pericarditis
Heiko J. Schmitt, M.D., Ph.D.
HJS
Outline
 Case
presentation
 Pericardial anatomy
 Clinical presentation and exam
 CT, MRI, and echocardiographic findings
 Hemodynamics
 Outcome after pericardectomy
HJS
Case Presentation - History
HJS
 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, 1014-9
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, 1014-9
Case Presentation - Initial Tests
HJS
 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, 1014-9
Case Presentation - Initial Tests
HJS
 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, 1014-9
Case Presentation - Final Tests


Mild cardiomegaly
increased interstitial markings

HJS
No pulmonary disease but
thickened pericardium
NEJM 2004, Vol 351, 1014-9
Case Presentation - Heart Catheter

HJS
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, 1014-9
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.
Pericardium - 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
HJS
Pericardium - Anatomy




HJS
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.
Constrictive Pericarditis - Etiology
Fibrinous
Hemorrhagic
Who develops constriction?
Purulent
HJS
Constrictive Pericarditis - Etiology
HJS
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
Constrictive Pericarditis - Pathophysiology





HJS
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
Constrictive Pericarditis - Clinic
Systemic venous
congestion
• Edema
• Abdominal
swelling and
discomfort 2nd to
ascites
•fullness, anorexia
Elevated left filling
pressure
• exertional dyspnea
• cough
• orthopnea
HJS
Decreased cardiac
output
• fatique
• muscle wasting
• poor exercise
tolerance
Braunwald, Heart Disease 4th ed., 1992
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) infrequently
found in constrictive pericarditis
Lancet 2002; 359, 1940-42
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, 1940-42
Constrictive Pericarditis - Exam




HJS
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
Constrictive Pericarditis - CXR







Braunwald, Heart Disease 4th ed., 1992
HJS
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).
Pulvaneswary: Constrictive Pericarditis, Australas.Radiol. 26:53, 1982
Constrictive Pericarditis - CT/MRI



HJS
May show thickened
pericardium
May exclude other
abnormalities.
Normal pericardium however
does not exclude restrictive
pericarditis.
Nishimura, Heart 2001, 86, 619-23
Constrictive Pericarditis - Echocardiography
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 - 2D Echo





HJS
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.
Constrictive 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
HJS
Constrictive Pericarditis - Doppler
a
HJS
v
x
y




High initial E velocity
short deceleration time
reduced velocity at atrial contraction
Decrease in E velocity during
inspiration



Corresponds with right atrial filling
Prominent a-wave
deep y-descent
Otto, Textbook of clinical Echocardiography, 3rd ed.
Constrictive Pericarditis - Echocardiography

A comprehensive echocardiogram may be considered diagnostic in
a subset of patients with classical findings
–
–
–
–

HJS
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
Constrictive Pericarditis - Catheterization
HJS
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
Constrictive Pericarditis - Catheterization


a
v


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
Grossman Cardiac catheterization, Angiography, and Intervention, 2000
6th edition
Constrictive Pericarditis - Catheterization





HJS
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
Constrictive Pericarditis - Catheterization


HJS
Increase of RA pressure during
inspiration
Kussmaul’s sign
Grossman Cardiac catheterization, Angiography, and Intervention, 2000
6th edition
Constrictive Pericarditis - Restrictive CM
HJS
Otto, Textbook of clinical Echocardiography, 3rd ed.
Constrictive Pericarditis - Restrictive CM


HJS
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
Constrictive Pericarditis - Mortality
Perioperative Mortality
15%
1980
11%
1990
5%
HJS
Etiology
 NYHA III-IV
 marked elevation of RV
end-diastolic pressure

2004
Braunwald, Heart Disease 4th ed., 1992
Constrictive pericarditis
Cause-specific survival after pericardectomy
HJS
Pericardectomy at the Cleveland
clinic foundation January1977December 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;43:1445-52
Constrictive pericarditis
Cause-specific survival after pericardectomy
HJS
Overall perioperative mortality
6.1%
Idiopathic
2.7%
Postsurgical
8.3%
Irradiation
21.4%
Miscellaneous
0%
J Am Coll Cardiol 2004;43:1445-52
Constrictive pericarditis
Cause-specific survival after pericardectomy



HJS
Idiopathic 88% 7-year
survival
postsurgical 66% 7-year
survival
irradiation 27% 7-year
survival
J Am Coll Cardiol 2004;43:1445-52
Constrictive 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.
HJS
Giessen, Germany
The Kids