The Hemodynamics of Restrictive Constrictive Cardiomyopathy Jad Skaf

Definition • Heart disease resulting in impaired ventricular filling. High diastolic pressures are required

Idiopathic (Familial) Restrictive Cardiomyopathy

Secondary Restrictive Cardiomyopathies • Infiltrative Amyloidosis Gaucher’s Hunter’s, Hurler’s • Storage disease Hemochromatosis Pompey

Constrictive Cardiomyopathy 1 -Cardiac Tamponade 2 -Constrictive pericarditis 3 -Effusive-constrictive pericarditis

Differentiation of Constriction vs. Restriction • • Similar clinical presentations Different etiologies Similar physical

Before Cath • HISTORY – Pericarditis, TB, CTD, Malignancy – Trauma CONSTRICTIVE – Amyloidosis,

• PHYSICAL Both exhibit Impaired Diastolic Filling: dyspnea, edema, fatigue, ascites… RHF –

ECHO Constriction • • Pericardial calcium Small LV, RV Dilated LA, RA Doppler: ventricular

ECHO RULES OUT • Systolic Dysfunction • Valvular Dysfunction • Peric. Effusion with early

VENTRICULAR FILLING PHYSIOLOGY RCM Pericardial Space Visceral Pericardium Impedance throughout Diastole Compliance Atrial filling

VENTRICULAR FILLING PHYSIOLOGY CP Early Diastole Mid-Diastole Normal Compliance Abrupt cessation of ventricular filling

Traditional Criteria: Constrictive Restrictive EDP equalisation LVEDP-RVEDP < 5 mm. Hg LVEDP-RVEDP > 5

Hurrell et al. Traditional Criteria: Constrictive Restrictive Sensitivity Specificity PPV NPV EDP equalisation LVEDP-RVEDP

Sharp et al. - 1960 Cardiac Tamponade Physiology

NORMAL PULMONARY WEDGE PRESSURE “E. F. G. ” INTRAPERICARDIAL PRESSURE i e INTRATHORACIC PRESSURE

Tamponade PULMONARY WEDGE PRESSURE “E. F. G. ” INTRAPERICARDIAL PRESSURE i e “E. F.

Constrictive Sensitivity Specificity PPV NPV EDP equalisation 60 38 4 57 PAP 93 24

Respiratory changes in the early diastolic transmitral pressure gradient as estimated by PCWP and

Respiratory changes in LVSP and RVSP Hurrell, D. G. et al. Circulation 1996; 93:

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The Hemodynamics of Restrictive & Constrictive Cardiomyopathy Jad Skaf, M. D. 11/02/2010

Definition • Heart disease resulting in impaired ventricular filling. High diastolic pressures are required to maintain cardiac output • Systolic function is usually normal • Presentation: LV or RV failure or biventricular HF

Idiopathic (Familial) Restrictive Cardiomyopathy

Secondary Restrictive Cardiomyopathies • Infiltrative Amyloidosis Gaucher’s Hunter’s, Hurler’s • Storage disease Hemochromatosis Pompey (glycogen) Fabry’s (glycolipid) • Endomyocardial Radiation-induced Eosinophilic syndromes Carcinoid heart disease • Inflammatory Sarcoidosis

Constrictive Cardiomyopathy 1 -Cardiac Tamponade 2 -Constrictive pericarditis 3 -Effusive-constrictive pericarditis

Differentiation of Constriction vs. Restriction • • Similar clinical presentations Different etiologies Similar physical exam signs Thick pericardium is not necessary or sufficient to make diagnosis of constriction • Overlapping echo and hemodynamic features • Important therapeutic implications

Before Cath • HISTORY – Pericarditis, TB, CTD, Malignancy – Trauma CONSTRICTIVE – Amyloidosis, Sarcoidosis RESTRICTIVE – Mantle radiation, cardiac surgery BOTH

• PHYSICAL Both exhibit Impaired Diastolic Filling: dyspnea, edema, fatigue, ascites… RHF – JVP • CP • RCM • TR with an enlarged compliant RA • RHF (pulm HTN, RV-MI) • Circulatory overload with systemic congestion – Kussmaul’s sign • RHF • Systemic venous congestion • Severe TR

ECHO Constriction • • Pericardial calcium Small LV, RV Dilated LA, RA Doppler: ventricular discordance • TDE: E’> 8 • PA syst us < 40 • Thick pericardium usual; no biopsy Restriction • • None Small LV , RV Dilated LA, RA Doppler: minimal respiratory variation • TDE: E’<7 • PA syst often > 40 • Pericardium not thickened; abnl biopsy

ECHO RULES OUT • Systolic Dysfunction • Valvular Dysfunction • Peric. Effusion with early tamponade physiology

VENTRICULAR FILLING PHYSIOLOGY RCM Pericardial Space Visceral Pericardium Impedance throughout Diastole Compliance Atrial filling at end of Diastole Parietal Pericardium

VENTRICULAR FILLING PHYSIOLOGY CP Early Diastole Mid-Diastole Normal Compliance Abrupt cessation of ventricular filling End Diastole -Fixed intracardiac volume -Ventricular Coupling -Pressure dissociation

CATH D LV RV

D D D D

Traditional Criteria: Constrictive Restrictive EDP equalisation LVEDP-RVEDP < 5 mm. Hg LVEDP-RVEDP > 5 mm. Hg High RVEDP/RVESP > 1/3 RVEDP/RVESP < 1/3 PAP PASP < 55 mm. Hg PASP > 55 mm. Hg Dip Plateau LV rapid filling wave> 7 mm. Hg LV rapid filling wave < 7 mm. Hg Kussmaul’s No Resp Var in mean RAP(<3) Resp Var in mean RAP (fall)

Hurrell et al. Traditional Criteria: Constrictive Restrictive Sensitivity Specificity PPV NPV EDP equalisation LVEDP-RVEDP < 5 mm. Hg EDP equalisation 60 PAP PASP < 55 mm. Hg PAP 93 High RVEDP/RVESP > 1/3 High RVEDP 93 Dip Plateau LV rapid filling wave> 7 mm. Hg Dip Plateau 93 Kussmaul’s No Resp Var in mean RAP(<3) Kussmaul’s 93 38 LVEDP-RVEDP > 5 mm. Hg 4 57 PASP > 55 mm. Hg 24 38 57 48 47 25 RVEDP/RVESP < 1/3 52 89 LV rapid filling wave < 7 mm. Hg 61 92 Resp Var in mean RAP (fall) 58 92

n=19 p<0. 05 Hurrell et al.

Respiratory Dynamic Criteria

Sharp et al. - 1960 Cardiac Tamponade Physiology

NORMAL PULMONARY WEDGE PRESSURE “E. F. G. ” INTRAPERICARDIAL PRESSURE i e INTRATHORACIC PRESSURE “E. F. G. ” = Estimated Filling Gradient

Tamponade PULMONARY WEDGE PRESSURE “E. F. G. ” INTRAPERICARDIAL PRESSURE i e “E. F. G. ” = Estimated Filling Gradient INTRATHORACIC PRESSURE

Hatle et al, 1989

Constrictive Sensitivity Specificity PPV NPV EDP equalisation 60 38 4 57 PAP 93 24 47 25 High RVEDP 93 38 52 89 Dip Plateau 93 57 61 92 Kussmaul’s 93 48 58 92 PCW-LV resp Gdt 93 81 78 94 LV/RV ID 100 95 94 100 Hurrell et al.

Respiratory changes in the early diastolic transmitral pressure gradient as estimated by PCWP and left ventricular (LV) minimum pressure n=36 n=15 p<0. 05 Hurrell, D. G. et al. Circulation 1996; 93: 2007 -2013

Respiratory changes in LVSP and RVSP Hurrell, D. G. et al. Circulation 1996; 93: 2007 -2013

Hatle et al, 1989

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