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

2. 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

3. Idiopathic (Familial) Restrictive Cardiomyopathy Restrictive Cardiomyopathy

4. 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

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

6. 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

7. Before Cath HISTORY –Pericarditis, TB, CTD, Malignancy – Trauma –Amyloidosis, Sarcoidosis –Mantle radiation, cardiac surgery Cath

8. 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

9. 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

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

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

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

13. CATH LV RV D

15. DDDDDDD

16. 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)

17. Traditional Criteria: Constrictive Restrictive Sensitivity Specificity PPV NPV EDP equalisation LVEDP-RVEDP 5 mmHg EDP equalisation 60 38 4 57 PAP PASP 55 mmHg PAP 93 24 47 25 High RVEDP RVEDP/RVESP > 1/3 RVEDP/RVESP < 1/3 High RVEDP 93 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 Hurrell et al.

18. n=19 p<0.05

19. Respiratory Dynamic Criteria

20. Cardiac Tamponade Physiology Sharp et al. - 1960

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

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

23. Hatle et al, 1989

25. 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 LV/RV ID Hurrell et al. 93 81 78 94 100 95 94 100

26. Hurrell, D. G. et al. Circulation 1996;93:2007-2013 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

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

28. Hatle et al, 1989

29. Thank you …