Constrictive Cardiomyopathy Constrictive Cardiomyopathy Versus Versus

Restrictive Cardiomyopathy Restrictive Cardiomyopathy EchocardiographyEchocardiography

Dr Djilali HanzalDr Djilali Hanzal

Cardiologist Cardiologist

National Guard Hospital National Guard Hospital

OutlineOutline

BackgroundBackground PhysiologyPhysiology Clinical FeaturesClinical Features Echocardiography :Echocardiography : M modeM mode 2D2D DopplerDoppler Tissue Doppler Tissue Doppler Strain ImagingStrain Imaging ConclusionConclusion

Etiology CP

Bertog SC, J Am Coll Cardiol. 2004;43(8):1445.

Tajik AJ Circulation. 1999;100(13):1380.

Symptoms

Varieties of constrictive pericarditis

Rien muller et al .J Thorac Imaging 1993

J Am Coll Cardiol 2004;43;1445-52

Anatomy Anatomy

Lt. Atrium Lt. Atrium is notis not Completely Completely intrapericardialintrapericardial

All other cardiac chambers All other cardiac chambers areare completely intrapericardialcompletely intrapericardial

Pulmonary Veins are Pulmonary Veins are completely intrathoraciccompletely intrathoracic

Effect of InspirationEffect of Inspiration

Normal PericardiumNormal PericardiumIntra thoracic pressureIntra thoracic pressure

Venous returnVenous return

Transient size of RVTransient size of RV

Normal LV fillingNormal LV filling

Constrictive PericarditisConstrictive Pericarditis Intra thoracic pressureIntra thoracic pressure

Venous returnVenous return

RV not expandedRV not expanded

Abnormal LV fillingAbnormal LV filling

Uptodate 2011

Mechanism

• FILLING FILLING IMPAIREMENTIMPAIREMENT

• LV-RV LV-RV INTERDEPENDANCEINTERDEPENDANCE

PhysiologyPhysiologyCP vs RCMCP vs RCM

Constrictive PericarditisMyocardial compliance is NLMyocardial compliance is NL

Pericardium not compliantPericardium not compliant

Septum compliantSeptum compliant

Rapid early diastolic Rapid early diastolic fillingfilling

cardiac volume is fixed by the cardiac volume is fixed by the pericardiumpericardium

Respiratory effect of LV Respiratory effect of LV on the RVon the RV

Restrictive Ab-Nl Myocardial complianceAb-Nl Myocardial compliance

Pericardium compliantPericardium compliant

Septum not compliantSeptum not compliant

Impedence to Impedence to fillingfilling increases increases throughout the diastolethroughout the diastole

No Respiratory effect of No Respiratory effect of RV and the LVRV and the LV

Restrictive Cardiomyopathy

(Myocardial Disorders)

Myocardial disease

Endomyocardial disease Storage disease

Infiltrative Noninfiltrative

Endomyocardial fibrosis

Hemochromatosis

AmyloidosisSarcoidosis

Idiopathic CMPDiabetic CMP

E William Hancok, Heart 2001, 86 343-349

Why is it important to make the distinction Why is it important to make the distinction RCM vs CP?RCM vs CP?

Associated with significant morbidity and Associated with significant morbidity and mortalitymortality

Restriction rarely treatable/curableRestriction rarely treatable/curable

Constriction may be curable with surgery. Constriction may be curable with surgery.

Inexplained CHF CXray: No Cardiomegaly ( Clinically, Cxray, BNP..)

Echo: Normal LV systolic function

Echo: Normal LV systolic function

Trans mitral Doppler: Restrictive Pattern: E/A>2Trans mitral Doppler: Restrictive Pattern: E/A>2

TDI:(E’>8cm/s, E/E’<15

Normal S wave)

TDI:(E’>8cm/s, E/E’<15

Normal S wave)

CPCP

TDI:E’<8cm/s,E/E’>15

TDI:E’<8cm/s,E/E’>15

RCMRCMCPCPCho YH and Schaff.Heart Fail Rev 2012

Inexplained CHF CXray: No Cardiomegaly ( Clinically, Cxray, BNP..)

Echo: M-Mode, 2-D Normal LV Systolic Function

M-mode and 2-DM-mode and 2-DCPCP

Pericardial thickening and calcificationPericardial thickening and calcificationSeptal bounceSeptal bounceDilated not collapsing Inferior Vena CavaDilated not collapsing Inferior Vena CavaFlattening of LV post wallFlattening of LV post wallEarly pathological outward and inward Early pathological outward and inward

movement of the IVSmovement of the IVSColor M-mode PropagationColor M-mode Propagation

18% of PC had normal thickness

CPCP

Differential Dx:Differential Dx: Constrictive PericarditisConstrictive Pericarditis Pericardial TamponadePericardial Tamponade Pulmonary HypertensionPulmonary Hypertension LBBBLBBB Right Ventricular PacingRight Ventricular Pacing

..

Paradoxal motion of the IVS Paradoxal motion of the IVS occurring in early diastoleoccurring in early diastole

Sensibility 62%,Specificity 93% Sensibility 62%,Specificity 93%

Journal of Thoracic Imaging. 27(1):w1, January 2012.

M-Mode CPM-Mode CP

Signs reflecting increased Signs reflecting increased ventricular interdependenceventricular interdependence

• Abrupt early diastolic anterior Abrupt early diastolic anterior motion of the IVS followed by a motion of the IVS followed by a rebound toward the LV post wall. rebound toward the LV post wall.

.Mastouri et al. Expert Rev Cardiovasc 2010

M-Mode CPM-Mode CP

Signs reflecting rapid early Signs reflecting rapid early ventricular diastolic filling:ventricular diastolic filling:

• Flattening at the LV post wallFlattening at the LV post wall

Sensitivity 92%, Specificity 100%

Voelkel et al ,Circulation. 1978 Nov;58(5):871-5.

Signs reflecting Signs reflecting increased Right Ventr increased Right Ventr diastolic pressure above diastolic pressure above Pulmonary Art pressurePulmonary Art pressure

• Premature opening of the Premature opening of the pulmonary valvepulmonary valve

Sensibility 14%,Specificity 100%

Mastouri et al. Expert Rev Cardiovasc 2010

M-Mode CPM-Mode CP

Sensibility 74%,Specificity 91%

Am J 2001,87,86-94

RCM 2-DRCM 2-D

Small LV cavity with Small LV cavity with large atrialarge atria

Increased wall Increased wall thickness ( especially thickness ( especially in interatrial septum in in interatrial septum in Amyloidosis)Amyloidosis)

Thickened valves and Thickened valves and granular sparkling granular sparkling texture (amyloidosis)texture (amyloidosis)

Inexplained CHF CXray: No Cardiomegaly ( Clinically, Cxray, BNP..)

Echo: M-Mode, 2-D Normal LV Systolic Function

Echo-Doppler:Restrictive Pattern: E/A>2,DT<150ms,IVRT<60ms

AV Inflow

Echo-DopplerEcho-Doppler

Mitral and Tricuspid InflowMitral and Tricuspid InflowIVRTIVRTTRTRHepatic Veins Hepatic Veins Pulmonary RegurgitationPulmonary RegurgitationPulmonary VeinsPulmonary VeinsSuperior Vena CavaSuperior Vena Cava

Specificity67%,

Sensibility 86%

J Am Coll Cardio 1994 jan.23,154-JACC,1994 Jan;23(1):154-62

CP

Constriction: Constriction: Non-respirophasicNon-respirophasic

Mixed Restriction and ConstrictionMixed Restriction and Constriction

Marked increase in PreloadMarked increase in Preload• Provocation test with head-up tilting or Provocation test with head-up tilting or

sitting position with decrease of the sitting position with decrease of the preload may unmask the CP.preload may unmask the CP.

Maisch, Seferovic, Ristic et al.ESC guidelines on pericardial disease, E J 2004

AF and CP

AF and CP

J Am Coll Cardio 2001;37:1936-42

CP

JACC 1994 Jan;23(1):154-62

Diagrammatic representation of the transmitral early (E-wave) and late (A-wave) velocities during diastole throughout the respiratory cycle.

Nihoyannopoulos P , Dawson D Eur J Echocardiogr 2009;10:iii23-iii33

Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2009. For permissions please email: journals.permissions@oxfordjournals.org

CP

CP

CP

Circulation 2002, Rajagopalan et al. AJC 2001

Specificity79%,Sensitivity 86%

Normal

CP

RCMCP

PV is RespirophasicPV is not Respirophasic

Normal

CP

CP vs COPDCP vs COPD

CP

Inexplained CHF CXray: No Cardiomegaly ( Clinically, Cxray, BNP..)

Echo: Normal LV systolic function

Echo-Doppler: Restrictive Pattern: E/A>2,DT<150ms,IVRT<60ms

AV Inflow

Tissue Doppler:Annular TDI

Specificity 89%,Sensibility100%

Rajagopalan et al .Am.J.Cardio 2001

E/e’=6

Am J Cardiol 2004;93:886-890

MITRAL “ANNULUS REVERSUS”MITRAL “ANNULUS REVERSUS”

E’ Lateral > E’ Septal

E’ Lateral< E’Septal

E’ Lateral =E’ Septal

Normal

CP

RCM

Reuss et al.Eur J Echocardiography 2009

Inexplained CHF CXray: No Cardiomegaly ( Clinically, Cxray, BNP..)

Echo: Normal LV systolic function

Echo-Doppler: Restrictive Pattern: E/A>2,DT<150ms,IVRT<60ms

AV inflow

Tissue Doppler:Annular TDI

Strain Imaging

Myocardial Mechanics in RCM and CPMyocardial Mechanics in RCM and CP

Deformation Parameter CP RCM

Longitudinal Strain

Normal

Circumferential Strain

Decreased

Decreased Normal

JACC Cardiovasc Imaging. 2008 Jan;1(1):29-38

2-D Speckle-tracking

CP

RCM

J Am Soc Echocardiogr 2009:22:24-33

CP

RCM

Em: Longitudinal early diastolic lengthening velocity J Am Soc Echocardiogr 2009:22:24-33

Too much for Diastology

ConclusionsConclusions

Dx has important therapeutic implicationsDx has important therapeutic implications

Clinical Presentaion similarClinical Presentaion similar

Echocardiography (Doppler,TDI, StrainEchocardiography (Doppler,TDI, Strain/Strain /Strain rate) have increased yield.rate) have increased yield.

Cardiac catheterisation still considered Cardiac catheterisation still considered mandatory.mandatory.

EndEnd

Inexplained CHF CXray: No Cardiomegaly ( Clinically, Cxray, BNP..)

Echo: Normal LV systolic function

Echo-Doppler: Restrictive Pattern: E/A>2,DT<150ms,IVRT<60ms

AV inflow

Tissue DopplerAnnular TDI

Strain Hemodynamic

Normal CP

QTDI

International J of Cardio 137(2009)22-39

RCM

International J of Cardio 137(2009)22-39

Major historical events in CPMajor historical events in CP

Korean Circ J 2012;42:143-150