Anatomy

Lt. Atrium is not completely intrapericardial

All other cardiac chambers are intrapericardial

Pulmonary Veins are completely intrathoracic

The pericardiumTwo-layered sac:

Inner serosal layer (visceral pericardium) adhering to outer wall of heart

Reflected at the level of the great vessels Joins the tough fibrous outer layer (parietal pericardium ).

A thin film of fluid (~ 50 ml).Pulmonary

Trunk

PL

VL

Function of pericardium1. Fixes heart within mediastinum and limits its motion; 2. Prevents extreme dilatation of heart during sudden rises

of intracardiac volume3. A barrier to limit spread of infection from the adjacent

lungs.

But patients with complete absence of the pericardium (congenitally or surgically) generally do fine without it? actual physiologic importance.

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Pathology of Constrictive PericarditisFibrotic, thickened, and adherent pericardium

restriction of diastolic filling of the heart.

An initial episode of acute pericarditis (may be subclinical): Organisation & resorption of effusionFibrous scarring and thickening of pericardiumObliteration of the pericardial space

Uniform restriction of filling of all heart chambers.

Calcium deposition stiffening of pericardium.

Scarring and loss of normal elasticity of pericardial sac §– restriction of ventricular filling in mid and late diastole

• majority of ventricular filling occurs rapidly in early diastole and • ventricular volume does not increase after the end of early filling period.

• Restrictive cardiomyopathy: – Non-dilated rigid ventricle – Severe diastolic dysfunction and restrictive filling

• Hemodynamics similar to CP.

Pathology of Constrictive Pericarditis

CP and Restrictive Cardiomyopathy• Both Share:

– diastolic dysfunction– Elevated diastolic pressures– abnormal ventricular filling– Decreased end diastolic volume (EDV).

Differentiation important Different management.Diagnosis:

Mostly: Clinical and conventional tests. Others: May need

Biopsy Surgical exploration

Pathophysiologic SimilaritySimilarity of:RestrictionRestriction and ConstrictionConstriction

MyocardialDisorder

PericardialDisorder

Abnormal increase in ventricular pressure impeding filling of RV & LV To NL filling of RV & LV To NL EDVEDV

Restriction Constriction

DifferencesDifferences

Constriction

Myocardial compliance is NL

RestrictionAb-Nl Myocardial compliance

No impedance to Diastolic EARLY FILLING

Total cardiac volume is fixed by Non-compliant pericardium

Impedance to filling increases throughout the diastole

Pericardium is compliantSeptum is non-compliant

Atria are able to empty into theventricles, though at higher Press.

proportional LV filling with atrial contraction Atrial enlargement

Marked Respiratory effect ofRV on LV

Minimal Respiratory effect of RV on LV

***********

Changing Etiology

Etiology of Constrictive PericarditisIdiopathic: ~ 50%Others:

Tuberculous: ~ 15% Post-viral pericarditis Post-surgical; Trauma Mediastinal Irradiation ESRD treated with HD Neoplastic pericardial infiltration Fungal and Parasitic P. Incomplete treatment of purulent

pericarditis Post MI pericarditis (Dressler

syndrome) Epicardial ICD patches implantation Pulmonary Asbestosis Methysergide Sarcoidosis

TB Chronic Constrictive Pericarditis

Importance of CP

Although uncommonPotential surgical cure. Last 15 years,

declining incidence of TB pericarditisincrease in therapeutic

mediastinal radiation and cardiac surgery.

********************

History• Prior history of

– pericarditis, – trauma, – cardiac surgery, or – a systemic disease (TB, connective tissue disease,

malignancy) CP

• A history of infiltrative disease that may involve the heart muscle (e.g., amyloidosis, sarcoidosis) RCMP

• Prior thoracic radiation treatment can result in either• constrictive pericarditis, • restrictive cardiomyopathy, or • Combination of both constrictive pericarditis and

restrictive cardiomyopathy.

Clinical Features - Symptoms and signs Reduced cardiac output:

fatigue, hypotension, reflex tachycardia

Elevated systemic venous pressure (Rt. Heart Failure) JVP distension, hepatomegaly with marked ascites and peripheral

edema.

Pulmonary venous congestion exertional dyspnea, cough and orthopnea

Chest pain typical of angina Under-perfusion of the coronary arteries or compression of an epicardial coronary artery by the thickened

pericardium.

Physical examination Elevated jugular venous pressure (JVP). ◊

JVP: deep, steep Y descent.

1. constrictive pericarditis, 2. restrictive cardiomyopathy, 3. TR with enlarged compliant RA, or 4. Rt. heart failure (e.g., RV infarction or PH).

Kussmaul's sign (lack of inspiratory decline in JVP) Pulsus paradoxus (rare in classic CP) Peripheral edema Ascites and hepatomegaly Pleural effusions. Pericardial knock (50%) in CP.

Not in restrictive cardiomyopathy. Audible S3: in RCMP; abrupt cessation of rapid ventricular filling.

Not usually present in CP.

Diagnostic Testing 2-D and Doppler Echo:

rule out other causes of right heart failure differentiation between CP and RMD may be difficult.

CT and MRI can help in detecting an abnormal pericardium, provide anatomical information But not pathophysiological abnormality.

Patients with surgically proven CP may have a normal-appearing pericardium on imaging studies (Talreja  D.R., Edwards  W.D., Danielson  G.K.;  et al. Circulation 108 2003

1852-1857).

Or Pericardial thickness without constriction: after radiation therapy or prior cardiac operation.

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Cardiac CatheterizationCardiac CatheterizationNot necessary for patients with typical CP:

Classic clinical presentation Typical features on noninvasive testing:

restrictive mitral inflow velocity, typical respiratory changes in transmitral and hepatic vein

Doppler velocities normal to increased (e’) mitral annular tissue velocity.

Indicated:If still a question of diagnosis after a comprehensive

Clinical Noninvasive evaluation.

Cardiac Cath. HemodynamicsHemodynamics Standard fluid-filled catheter Standard fluid-filled catheter

systems:systems: Pressure data is sufficient for

most clinical hemodynamic studies

Many artifacts and suboptimal frequency-response to accurately assess ventricular properties in research studies.

High-Fidelity High-Fidelity

Micromanometers: Micromanometers: § Useful for studies of Cardiac

Mechanics Combined with quantitative

volume measurements to examine chamber function.

Volumetric data acquired by: ventricular angiography, simultaneous echocardiography.

MeasurementsObtain Rt. and Lt. cardiac pressure waveforms. All right-sided pressure recorded simultaneously with LV

pressure.

Pressures recorded during: normal quiet respiration (for measurement of end-expiratory pressures)

and exaggerated respiration

Volume loading of 1 liter NS if on diuretics and RAP (<15 mm Hg).

Overdrive pacing if in A. Fib

RAP, PASP, RVEDP, PCWP, LVEDP, & height of rapid filling wave (RFW).

These measurments are only Possible using High-fidelityMicromanometer systems .

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Traditional Criteria for CP1. Diastolic equalization of Pressures:

LVED – RVEDP ≤ 5 mm Hg

2. Narrow RV Pulse Pressure: RVEDP/RVSP > 1/3 (33%)

3. Lack of significant pulmonary HTN: SPAP < 55 mm Hg

4. Dip and Plateau Pressure (Square Root Sign): Height of LV rapid filling wave (RFW) > 7 mm Hg

5. Kussmaul’s Sign: Inspiratory fall in mean RA pressure < 3 mm Sometime elevation of mean RA pressure with

inspiration

1- RV and LV Tracings1- RV and LV Tracings In diastole:

1) RV & LV diastolic pressures- Elevated - Equalized

4) Square root sign Rapid filling wave (RFW)

due to tithering effect of pericardium, pulling the ventricular muscle back to its diastolic configuration

Muscle is compliant steep drop in early diastole

Because of restraint RFW, then plateau

2) Pulse Pressure of RV > 1/3 «

In Systole: 3) SRVp < 55 mmHg

RV SP=60RV DP=30RVDP/RVSP=30/60=1/2 (50%) (>1/3; >33%)

RV, LV pressure tracings

Stawowy P et al. Circ Cardiovasc Imaging 2008;1:173-174

- rapid pressure deceleration (dip) §- rapid filling wave (RFW) - pressure equalization of RV and LV (plateau)

Square Root Sign

WhatDoes

it say?

Stawowy P et al. Circ Cardiovasc Imaging 2008;1:173-174

av

c

2- RA pressure tracings 2- RA pressure tracings

Elevated RA pressure

Sharp, deep Y descent

RA and LV RA and LV pressurespressures

RA and LV represent inflow and outflow of heart

In CP: < 5 mm Hg difference in

diastole

Sometimes: Steep X and Y descents “W” shape of RA tracing

x y

a v a

Kussmaul SignKussmaul Sign Deep inspiration:

- ve pressure in intrathoracic IVC

+ ve pressure in intraabdominal IVC PG in IVC pulling blood to chest.

Normal pericardium allows transmission of this PG to cardiac chambers flow to RA.

In CP: less transmission of – ve pressure less drop in RA pressure in inspiration < 3 mmHg

(5)

?

Hepato-jugular Reflux

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3- LV and PCWP Tracing3- LV and PCWP TracingDissociationDissociation of Intrathoracic and Intracardiac Pressures

Hatle LK, et. al.

Circ. 1989;79357-370

v vv

v

3- LV and PCWP Tracing3- LV and PCWP Tracing

Catheter in PA:Outside heart Pressure changes

with respiratory variation preserved (PA & PCWP).

Intracardiac (LV) p less changes Drop in PCWP-LV

diastolic PG during inspiration flow into LV.

Insp. Exp.

Dynamic, Respiratory Variations in CPDynamic, Respiratory Variations in CP In Inspiration:

dissociation of intrathoracic and intracardiac pressures increase in ventricular interaction Increasing RV filling filling Decreasing LV fillingfilling.

Alternative hypothesis for discordance: increase in inspiratory flow to RV decreased transseptal gradient

decrease in early diastolic suctionsuction of LV.

Doppler Echo: transmitral and hepatic vein flows. §

PCWP and LV- RestrictionRestriction

Simultaneous drop in LVDP and PCWP with respiration Negative intrathoracic pressure is transmitted to Intracardiac chambers

?

4- RV and LV pressure Tracing4- RV and LV pressure Tracing

Systolic LV & RV pressure Respiratory DiscordanceRespiratory Discordance

Insp. Exp.Insp. Coupling

Exp. De-Coupling

Ventricular Interdependence During Respirations Constrictive Pericarditis vs. Restrictive Cardiomyopathy

Constrictive Pericarditis(LV and RV discordant)

Restrictive Cardiomyopathy(LV and RV concordant)

Hurrell et al, Circulation 1996; 93:2007

Effect of Inspiration: ConstrictionSimultaneous Echo and Cath DataSimultaneous Echo and Cath Data

PCWP

Inspir.

No proportionate decrease in LV diastolic pressure

Inspir. Expir.

Expir.Insp.

Expir.

Decreased transmitral gradient Transmitral flow

RV SV LV SV

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Constrictive Pericarditis in the Modern Era: Novel Criteria for Diagnosis in the Cardiac Catheterization Laboratory

Criterion Sensitivity (%)

Specificity (%)

Positive Predictive

Accuracy (%)

Negative Predictive

Accuracy (%)

LVEDP − RVEDP ≤5

mm Hg46 54 58 40

PASP <55 mm Hg

90 29 73 66

RVEDP/RVSP

>1/3 93 46 71 79

LV RFW >7 mm Hg

45 44 62 42

Inspiratory decrease in RAP

<5 mm Hg71 37 62 39

Systolic area index >1.1 97 100 100 95

J Am Coll Cardiol. 2008;51(3). Deepak R. Talreja,; Rick A. Nishimura,; Jae K. Oh,; David R. Holmes

J Am Coll Cardiol. 2008;51(3):315-319. doi:10.1016/j.jacc.2007.09.039

≤ 5

≤ 55

> 33%

> 7

Pressure Waveforms During Exaggerated RespirationThe area under ventricular pressure curve

was used to determine change in relative volumes of LV and RV

a better determinant of beat-to-beat stroke volume than the peak pressure alone

The systolic area index: RV area (mm Hg × s) to LV area (mm Hg × s) in inspiration versus expiration.

LV and RV Pressure From 2 Patients During Expiration and Inspiration

(A) constrictive pericarditis. During inspiration:

1. increase in area of RV pressure curve (orange) compared with expiration.

2. Enhancement of LV-RV coupling.

3. area of LV pressure curve (yellow) decreases in height and width as compared with expiration.

B) restrictive myocardial disease During inspiration:

1. decrease in area of RV pressure curve (orange) as compared with expiration.

2. area of the LV pressure curve (yellow) is unchanged as compared with expiration.

CP

RP

LV-RV coupling

1

2

3

1

2 2

J Am Coll Cardiol. 2008;51(3):315-319. doi:10.1016/j.jacc.2007.09.039

Scatterplot of the Ratio of RV to LV Area Ratio of RV to LV Area Comparing Expiration Versus Inspiration.

This ratio is a measurement of the degree of ventricular interaction.

1.1

Systolic area index

>1.1 97 100 100 95

RA AngiogramAbnormal contour of

RAAttenuated convex

edgeThickening of heart

border

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ManagementManagementCCP is a progressive irreversible disease

Minority survive for years with modest elevated JVP and peripheral edema controlled by diet and diuretics.

Drugs that slow HR, eg beta blockers and Ca2+ channel blockers should be avoided as mild sinus tachycardia is a compensatory mechanism.

The majority of patients become progressively more disabled and subsequently suffer the complications of severe cardiac cachexia.

Surgical & Pathology Findings in CP obliteration of pericardial space bulging of heart upon incision of pericardium, abnormal pericardial thickening and/or calcificationpost-operatively

- decrease in RA - increase in cardiac index.

Rare clinical syndromePericardial effusion and pericardial constriction, Constrictive hemodynamics persistent after p. effusion is

removed.Mechanism:

visceral pericardial constriction Visceral pericardiectomyPericardial effusions vary in size and age.Type: Transudative, exudative, sanguineous, or chylous.An effusion for months to years may evolve into ECP

Any point of time; from occurrence of P. Effusion to development of constriction.

Symptoms due to limitation of end-diastolic volumeend-diastolic volume. - pericardial effusion/ tampnadetampnade, And - pericardial

constriction.

EFFUSIVE CONSTRICTIVE PERICARDITISEFFUSIVE CONSTRICTIVE PERICARDITIS(ECP)(ECP)

EFFUSIVE CONSTRICTIVE PERICARDITISEFFUSIVE CONSTRICTIVE PERICARDITISEtiology

Idiopathic factors IrradiationCardiac surgeryNeoplasm - Most commonly lung, breast, or hematologic Infectious disease - Particularly in immunocompromised states

most commonly tuberculosis and fungal disease, Streptococcus species reported

Myocardial infiltrationConnective tissue diseaseUremia

Hancock in 1960: helped in current understanding of ECP. Hancock, EW. Subacute effusive-constrictive pericarditis. Circulation 1971. Hancock, EW. On the elastic and rigid forms of constrictive pericarditis. Am Heart J

1980.

24 patients undergoing pericardiectomy for CP, 9 had concurrent effusion. 6 of 9 had hemodynamic studies before surgery.

Sagrista-Sauldea et al in 2004; prospective study of 1184 patients with pericarditis,

6.9% of 218 patients with tamponade had confirmed EFP. Sagrista-Sauleda J, Angel J, Sanchez A, et al. Effusive-constrictive pericarditis. N Engl J Med. 2004

EFFUSIVE CONSTRICTIVE PERICARDITIS EFFUSIVE CONSTRICTIVE PERICARDITIS (ECP)(ECP)

Clinical clues to ECP:1. Pulsus paradoxus

rare in classical CP (absence of transmission of the inspiratory decline in pressure to right heart chambers)  

2.  Absence of pericardial knock (effusion)3. The Y descent less dominant than expected4. Kussmaul's sign frequently absent

PA and Ao. TracingPA and Ao. Tracing

SPAP> 55 mm HgPulsus Paradoxus

(Ao.)

Pulsus Paradoxus

Insp. Exp.

?

EFFUSIVE EFFUSIVE CONSTRICTIVECONSTRICTIVE PERICARDITIS PERICARDITIS(ECP)(ECP)

Diagnosis:during pericardiocentesis in patients considered to

have uncomplicated cardiac tamponade.

Despite lowering pericardial pressure to normal, 1. persistence of elevated RA pressure

2. development of y decent dominance

3. impaired respiratory variation. §

because of visceral constrictive component of the syndrome persistent elevation and equalization of intracardiac diastolic pressures.

Y

Persistently elevated RA pressure after pericardiocentesis

Cardiac tamponade Cardiac tamponade complicating right heart failure or tricuspid regurgitation.

ConclusionCP still challenging diagnosis for clinicians, especially

when both myocardial and pericardial disease present.

Although noninvasive testing help in the diagnosis of CP, some cases remain unclear.

Dynamic respiratory changes reflecting the enhancement of ventricular interaction at cardiac catheterization is most useful.

The ratio of RV to LV systolic pressure X area during inspiration vs. expiration (systolic area index) is a novel measurement of enhanced ventricular interaction.

Cardiac CatheterizationTypical hemodynamic response in CP:

early rapid filling and equalization of end-diastolic pressures in all 4 cardiac chambers but also in RMD.

More severe pulmonary hypertensiongreater difference between LVEDP and RVEDP

in patients with RMD. Though statistically significant differences in overall

values of these criteria, but the – ve and + ve predictive value were of limited.

Pressure Waveforms During Exaggerated Respiration Peak inspiratory beat was selected as the systolic impulse preceded by the lowest

early diastolic nadir of the LV pressures. Selection of the peak inspiratory beat required that the early diastolic nadir was at a

minimum for the diastolic filling period before and after the systolic pressure contours. Peak expiratory beat was selected as the systolic impulse that was preceded by the

highest early diastolic nadir of the LV pressure.

In CP: inspiratory decrease in the LV volume and enhancement of ventricular coupling (obligatory increase in RV volume) The LV pressure curves become smaller in terms of both the height and width of

curve, The RV pressure curve becomes larger during peak inspiration.

Previously, RV index based on RV peak systolic pressure variation between inspiration and expiration was used as a measure of ventricular coupling.

We subsequently found that changes in the peak pressure alone were not sensitive enough to detect all patients with CP.

Therefore, the area under the ventricular pressure curve was used to determine the change in the relative volumes of the LV and RV

a better determinant of beat-to-beat stroke volume than the peak pressure alone The systolic area index: the ratio of RV area (mm Hg × s) to LV area (mm Hg × s)

in inspiration versus expiration.

Constrictive Pericarditis in the Modern EraNovel Criteria for Diagnosis in the Cardiac Catheterization Laboratory

Deepak R. Talreja, MD, FACC; Rick A. Nishimura, MD, FACC; Jae K. Oh, MD, FACC; David R. Holmes, MD, FACCJ Am Coll Cardiol. 2008;51(3):315-319. doi:10.1016/j.jacc.2007.09.039

Abstract Objectives  This study sought to determine the clinical utility of a new catheterization

criterion for the diagnosis of constrictive pericarditis (CP). Background  The finding of early rapid filling and equalization of end-diastolic

pressures obtained by cardiac catheterization are necessary for the diagnosis of CP, but these findings are also present in patients with restrictive myocardial disease (RMD). Enhanced ventricular interaction is unique to CP.

Methods  High-fidelity intracardiac pressure waveforms from 100 consecutive patients undergoing hemodynamic catheterization for diagnosis of CP versus RMD were examined. Fifty-nine patients had surgically documented CP and comprised group 1; the remaining 41 patients with RMD comprised group 2. The ratio of the right ventricular to left ventricular systolic pressure-time area during inspiration versus expiration (systolic area index) was used as a measurement of enhanced ventricular interaction.

Results  There were statistically significant differences in the conventional catheterization criteria between CP and RMD, but the predictive accuracy of any of the criteria was <75%. The systolic area index had a sensitivity of 97% and a predictive accuracy of 100% for the identification of patients with surgically proven CP.

Conclusions  The ratio of right ventricular to left ventricular systolic area during inspiration and expiration is a reliable catheterization criterion for differentiating CP from RMD, which incorporates the concept of enhanced ventricular interdependence.