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

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 Definition

Idiopathic (Familial) Restrictive Cardiomyopathy 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 –Amyloidosis, Sarcoidosis –Mantle radiation, cardiac surgery Cath

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

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

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

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

VENTRICULAR FILLING PHYSIOLOGY CP Early DiastoleEnd DiastoleMid-Diastole Normal ComplianceAbrupt cessation of ventricular filling -Fixed intracardiac volume -Ventricular Coupling -Pressure dissociation

CATH LV RV D

DDDDDDD

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

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

n=19 p<0.05

Respiratory Dynamic Criteria

Cardiac Tamponade Physiology Sharp et al

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

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

Hatle et al, 1989

Constrictive Sensitivity Specificity PPV NPV EDP equalisation PAP High RVEDP Dip Plateau Kussmaul’s PCW-LV resp Gdt LV/RV ID Hurrell et al

Hurrell, D. G. et al. Circulation 1996;93: Respiratory changes in the early diastolic transmitral pressure gradient as estimated by PCWP and left ventricular (LV) minimum pressure n=36n=15 p<0.05

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

Hatle et al, 1989

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