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

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.


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


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


HJSConstrictive Pericarditis - Clinic


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


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


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.


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


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


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


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


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



Increase of RA pressure during inspiration

Kussmaul’s sign


HJSConstrictive Pericarditis - Restrictive CM


HJSConstrictive Pericarditis - Restrictive CM

Ventricular interdependence not seen in restrictive cardiomyopathy Discordant change in left and right peak systolic pressure with

repiratory changes.


HJSConstrictive Pericarditis - Mortality

Etiology NYHA III-IV marked elevation of RV

end-diastolic pressure


11%

15%

5%

Perioperative Mortality

1980

1990

2004

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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

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Overall perioperative mortality 6.1%

Idiopathic 2.7%

Postsurgical 8.3%

Irradiation 21.4%

Miscellaneous 0%

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

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