Constrictive Cardiomyopathy Versus Restrictive Cardiomyopathy Echocardiography Dr Djilali

Constrictive Cardiomyopathy Versus Restrictive Cardiomyopathy Echocardiography Dr Djilali Hanzal Cardiologist National Guard Hospital

Outline q Background q Physiology q Clinical Features q Echocardiography : § M mode § 2 D § Doppler § Tissue Doppler § Strain Imaging q Conclusion

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

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

Varieties of constrictive pericarditis Rien muller et al. J Thorac Imaging 1993

J Am Coll Cardiol 2004; 43; 1445 -52

Anatomy Lt. Atrium is not Completely intrapericardial All other cardiac chambers are completely intrapericardial Pulmonary Veins are completely intrathoracic

Effect of Inspiration Normal Pericardium Intra thoracic pressure Venous return Constrictive Pericarditis q Intra thoracic pressure q Venous return Transient size of RV q RV not expanded Normal LV filling q Abnormal LV filling Uptodate 2011

Mechanism • FILLING IMPAIREMENT • LV-RV INTERDEPENDANCE

Physiology CP vs RCM Constrictive Pericarditis Myocardial compliance is NL Pericardium not compliant Septum compliant Rapid early diastolic filling cardiac volume is fixed by the pericardium Respiratory effect of LV on the RV Restrictive Ab-Nl Myocardial compliance Pericardium compliant Septum not compliant Impedence to filling increases throughout the diastole No Respiratory effect of RV and the LV

Restrictive Cardiomyopathy (Myocardial Disorders) Myocardial disease Endomyocardial disease Storage disease Endomyocardial fibrosis Infiltrative Noninfiltrative Amyloidosis Sarcoidosis Idiopathic CMP Diabetic CMP Hemochromatosis E William Hancok, Heart 2001, 86 343 -349

Why is it important to make the distinction RCM vs CP? q Associated with significant morbidity and mortality q Restriction rarely treatable/curable q Constriction may be curable with surgery.

Inexplained CHF CXray: No Cardiomegaly ( Clinically, Cxray, BNP. . ) Echo: Normal LV systolic function FINDINGS Trans mitral Doppler: Restrictive Pattern: E/A>2 TDI: (E’>8 cm/s, E/E’<15 Normal S wave) CP TDI: E’<8 cm/s, E/E’>15 CP RCM Cho YH and Schaff. Heart Fail Rev 2012

Inexplained CHF CXray: No Cardiomegaly ( Clinically, Cxray, BNP. . ) Echo: M-Mode, 2 -D Normal LV Systolic Function Findings

M-mode and 2 -D CP q Pericardial thickening and calcification q Septal bounce q Dilated not collapsing Inferior Vena Cava q Flattening of LV post wall q Early pathological outward and inward movement of the IVS q Color M-mode Propagation

18% of PC had normal thickness

CP q Differential Dx: § § § Constrictive Pericarditis Pericardial Tamponade Pulmonary Hypertension LBBB Right Ventricular Pacing . q Paradoxal motion of the IVS occurring in early diastole § Sensibility 62%, Specificity 93% Journal of Thoracic Imaging. 27(1): w 1, January 2012.

M-Mode CP • Signs reflecting increased ventricular interdependence Abrupt early diastolic anterior motion of the IVS followed by a rebound toward the LV post wall. Mastouri et al. Expert Rev Cardiovasc 2010.

M-Mode CP q Signs reflecting rapid early • ventricular diastolic filling: Flattening at the LV post wall q Sensitivity 92%, Specificity 100% Voelkel et al , Circulation. 1978 Nov; 58(5): 871 -5.

M-Mode CP q Signs reflecting increased Right Ventr diastolic pressure above Pulmonary Art pressure • Premature opening of the pulmonary valve § Sensibility 14%, Specificity 100% Mastouri et al. Expert Rev Cardiovasc 2010

Sensibility 74%, Specificity 91% Am J 2001, 87, 86 -94

RCM 2 -D q Small LV cavity with large atria q Increased wall thickness ( especially in interatrial septum in Amyloidosis) q Thickened valves and granular sparkling 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<150 ms, IVRT<60 ms AV Inflow Findings

Echo-Doppler q Mitral and Tricuspid Inflow q IVRT q TR q Hepatic Veins q Pulmonary Regurgitation q Pulmonary Veins q Superior Vena Cava

CP Specificity 67%, Sensibility 86% JACC, 1994 Jan; 23(1): 154 -62 J Am Coll Cardio 1994 jan. 23, 154 -

Constriction: Non-respirophasic Mixed Restriction and Constriction Marked increase in Preload • Provocation test with head-up tilting or sitting position with decrease of the 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: iii 23 -iii 33 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

Normal CP Specificity 79%, Sensitivity 86% Circulation 2002, Rajagopalan et al. AJC 2001

CP

Normal CP PV is Respirophasic RCM PV is not Respirophasic

CP

CP vs COPD CP

Inexplained CHF CXray: No Cardiomegaly ( Clinically, Cxray, BNP. . ) Echo: Normal LV systolic function Echo-Doppler: Restrictive Pattern: E/A>2, DT<150 ms, IVRT<60 ms AV Inflow Tissue Doppler: Annular TDI Findings

Specificity 89%, Sensibility 100% Rajagopalan et al. Am. J. Cardio 2001

E/e’=6 Am J Cardiol 2004; 93: 886 -890

MITRAL “ANNULUS REVERSUS” Normal E’ Lateral > E’ Septal CP E’ Lateral< E’Septal RCM E’ Lateral =E’ Septal 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<150 ms, IVRT<60 ms AV inflow Tissue Doppler: Annular TDI Strain Imaging Findings

Myocardial Mechanics in RCM and CP Deformation Parameter Longitudinal Strain Circumferential Strain CP Normal Decreased JACC Cardiovasc Imaging. 2008 Jan; 1(1): 29 -38 RCM Decreased Normal

CP RCM 2 -D Speckle-tracking 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

Conclusions q Dx has important therapeutic implications q Clinical Presentaion similar q Echocardiography (Doppler, TDI, Strain/Strain rate) have increased yield. q Cardiac catheterisation still considered mandatory.

End

Inexplained CHF CXray: No Cardiomegaly ( Clinically, Cxray, BNP. . ) Echo: Normal LV systolic function Echo-Doppler: Restrictive Pattern: E/A>2, DT<150 ms, IVRT<60 ms AV inflow Tissue Doppler Annular TDI Hemodynamic Strain

QTDI Normal CP International J of Cardio 137(2009)22 -39

RCM International J of Cardio 137(2009)22 -39

Major historical events in CP Korean Circ J 2012; 42: 143 -150