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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
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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
Restrictive Cardiomyopathy 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
Cath Before Cath • HISTORY • Pericarditis, TB, CTD, Malignancy – Trauma • Amyloidosis, Sarcoidosis • Mantle radiation, cardiac surgery CONSTRICTIVE RESTRICTIVE BOTH
Both exhibit Impaired Diastolic Filling: dyspnea, edema, fatigue, ascites… RHF • 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
ECHO 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 Constriction Restriction
ECHO RULES OUT Systolic Dysfunction Valvular Dysfunction Peric. Effusion with early tamponade physiology
VENTRICULAR FILLING PHYSIOLOGY RCM Pericardial Space Parietal Pericardium Visceral Pericardium Impedance throughout Diastole Compliance Atrial filling at end of Diastole
VENTRICULAR FILLING PHYSIOLOGY CP Early Diastole Mid-Diastole End Diastole Normal Compliance Abrupt cessation of ventricular filling -Fixed intracardiac volume -Ventricular Coupling -Pressure dissociation
D CATH LV RV
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Traditional Criteria: Constrictive Restrictive EDP equalisation LVEDP-RVEDP < 5 mmHg LVEDP-RVEDP > 5 mmHg High RVEDP RVEDP/RVESP > 1/3 RVEDP/RVESP < 1/3 PAP PASP < 55 mmHg 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: Hurrell et al. Constrictive Restrictive Sensitivity Specificity PPV NPV EDP equalisation LVEDP-RVEDP < 5 mmHg LVEDP-RVEDP > 5 mmHg EDP equalisation60 38 4 57 PAP PASP < 55 mmHg PASP > 55 mmHg PAP 93 24 47 25 High RVEDP RVEDP/RVESP > 1/3 RVEDP/RVESP < 1/3 High RVEDP93 38 52 89 Dip Plateau LV rapid filling wave> 7 mmHg LV rapid filling wave < 7mmHg Dip Plateau 93 57 61 92 Kussmaul’s No Resp Var in mean RAP(<3) Resp Var in mean RAP (fall) Kussmaul’s 93 48 58 92
n=19 p<0.05 Hurrell et al.
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 INTRATHORACIC PRESSURE i e “E.F.G.” = Estimated Filling Gradient
Constrictive Sensitivity Specificity PPV NPV EDP equalisation60 38 4 57 PAP 93 24 47 25 High RVEDP93 38 52 89 Dip Plateau 93 57 61 92 Kussmaul’s 93 48 58 92 PCW-LV resp Gdt LV/RV ID 93 81 78 94 100 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