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Restriction and Constriction Nick Tehrani, MD Restrictive Cardiomyopathy EVOLVED As a bedside clinical diagnosis of constriction confirmed by right heart catheterization findings of constrictive physiology in patients who: POROVED NOT TO HAVE ANY PERICARDIAL DISEASE Traditional Hemodynamic Criteria Traditional Hemodynamic Criteria Utilitity Traditionalof: Hemodynamic Criteria: of LIMITED Utility Traditionalare Hemodynamic Criteria Hurrell DG, Nishimura RA, Higano ST, Appelton CP, Danielson GK, Holmes DR, Tajik AJ. Value of dynamic respiratory changes in left and right ventricular pressures for the diagnosis of constrictive pericarditis. Circ. 1996; 93:2007-2013 Restrictive Cardiomyopathy Represents an extreme form of Diastolic Dysfunction: Abnormal increase in Diastolic ventricular pressure impeding filling of the LV To NL EDV Diastole: A historical view Diastole as the passive interval between systolic events Discovery of Frank-Starling mechanism: LV-EDV, helps regulate the SV Katz: After MV opening, LV pressure continue to decline, despite LV volume LV as an active suction incresase pump in early diastole. Diastolic Properties of the LV End of IVRT Active suction. ATP req’d To Re-uptake Ca++ Quantitative assessent of the 4 phases of Diastole: Late diastolic filling IVRT Early diastolic filling Diastasis IVRT>100 ms Earliest diastolic abnormality. Impaired LV relax. Filling pressures=NL Dz. progression: Decreased LV compliance, and Increased filling pressure Quantitative assessment of the 4 phases of Diastole: IVRT Early filling: DT < 130-180 ms Interplay of Early and Late filling: E:A ratios……. If E at A> 20 cm/s => E/A unreliable Utilized in relation to PV “a” wave duration. Tachycardia PR prologgation “A” wave duration……. E:A ratios, as a Function of Age Impaired LV relaxation (IVRT ) Decreased LV Compliance Pseudonormal Restrictive The three abnormal LV filling patterns Pathophysiologic Similarity of: Restriction and Constriction Abnormal increase in ventricular pressure impeding filling of the LV To NL EDV Restriction Myocardial Disorder Constriction Pericardial Disorder Anatomy Lt. Atrium is not completely intrapericardial All other cardiac chambers are completely intrapericardial Pulmonary Veins are completely intrathoracic Effect of Inspiration Normal Pericardium: Inspiratory decrease in intrathoracic pressure is uniformly transmitted to the lungs, PVs, LA, LV, RA, and RV Effect of Inspiration Constrictive Pericarditis: Thickened pericardium isolates the heart form transmission of intrathoracic pressure changes Increased inspiratory capacitance of the Lungs PVs, and LA => PCWP decrease BUT The decrease in intrathoracic pressure is not transmitted to the LV, RV, RA Dissociation of Intrathoracic and Intracardiac Pressures First demonstrated to be present in constrictive pericarditis using Doppler techniques in 1989, by Hatle in her landmark study. Hatle LK, Appleton CP, Popp RL. Differentiation of constrictive pericarditis And restrictive cardiomyopathy by Doppler Echocardiography. Circ. 1989;79357-370 Dissociation of Intrathoracic and Intracardiac Pressures The inciting Physiologic Event. Hatle LK, et. al. Circ. 1989;79357-370 Ventricular Interdependence Hatle LK, et. al. Circ. 1989;79357-370 Insp Expir Ventricular Pressures Are DISCORDANT Traditional v.s. Dynamic Catheterization Hemodynamics Why bother with Echo These measurments aregiven only The greatusing utilityHigh-fidelity of Dynamic Possible Respiratory cath measurments? Micromanometer systems (not a common practice). Dissociation of Intrathoracic and Intracardiac Pressures Effect of Inspiration: Constriction Inspir. Insp. PCWP Expir. PCWP PCWP Expir. Expir. Inspir. No proportionate decrease in LV diastolic pressure Decreased transmitral gradient => Transmitral flow LV SV RV SV Pathophysiologic Differences Constriction Restriction Myocardial compliance is NL No impedence to Ab-Nl Myocardial compliance Diastolic EARLY FILLING Total cardiac volume is fixed by the pericardium Atria are able to empty into the Ventricles, though at higher Press. Marked Respiratory effect of LV on the RV Impedence to filling increases throughout the diastole Pericardium is compliant Septum is non-compliant Reduction of the proportion of LV filling with atrial contraction: => Atrial enlargement Minimal Respiratory effect of RV on the LV Specific Echocardiographic Criteria for Constriction/Restriction Mitral E wave pattern Pulmonary Vein pattern Hepatic Vein pattern Mitral E wave Criteria for Constriction Decrease in of 25% in Mitral “E” velocity on inspiration. In RESTRICTION: no respiratory variation of Mitral inflow There is Specific Echocardiographic Criteria for Constriction/Restriction Mitral E wave pattern Pulmonary Vein pattern Normal PV Flow-TTE PSV1- LA relaxation and pressure decrease. PSV2- Interaction of RVSV, w/ LA pressure and compliance. Utilized in relation to Mitral “A” wave duration. PVa duration- Interplay of multiple factors Three abnormal PV filling patterns in Restriction E:A ratios, as a function of Age Impaired LV relaxation (IVRT ) Decreased LV Compliance Pseudonormal Restrictive Relation of Mitral “A” wave to Pulmonary Venous “a” wave duration Normal Physiology With LA contraction Forward flow Volume and Duration Exceeds Backward flow into the PV Relation of Mitral “A” wave to PV “a” wave duration Restrictive Physiology: PV-a Velocity > 35 cm/s OR PV-a duration, 30 ms longer than Mitral “A” wave duration. 200 ms 121 ms PV interrogation using TTE is often techniaclly limited. PVs are best assessed using TEE Normal PV Flow-TEE Rt. Upper PV NO Variation from Inspiation to Expiration LV inflow PV Dopplar Patterns in Restriction-TEE Lt. Upper PV LV Inflow PV flow is not respirophasic Systolic/Diastolic velocity is markedly down in both inspiration and expiration LV inflow Peak-E velocity is not respirophasic PV Dopplar Patterns in Costriction-TEE Lt. Upper PV PV flow LV Inflow IS Respirophasic: 25% variation of both the Systolic and Diastolic components Systolic/Diastolic Ratio higher than for restriction (0.7 v.s. 0.4) LV inflow Peak-E: 17% respiratory variation (v.s. none for restriction) Specific Echocardiographic Criteria for Constriction/Restriction Mitral E wave pattern Pulmonary Vein pattern Hepatic Vein pattern Respiratory Cycle :Hepatic Vein Flow IVC Inspiration Expiration Hepatic Vein Dopplar: Normal Normal Systolic and diastolic forward flow S-vel. > D-vel. Diastolic flow reversal: Expir.>>Insp. Hepatic Vein Dopplar: Constriction Constriction Diastolic flow reversal is augmented in expiration. DFRexp.>25% forward diastolic velocity Hepatic Vein Dopplar: Restriction Restriction Forward flow primarily in Diastole. Inspiration increases both >systolic, and >Diastolic Flow reversals. Nasser S Tehrani: These pts not respond as well to surgery Hepatic Vein Dopplar: Compilation Mixed physiology (restriction/constriction) Diastolic flow reversal during both Ispiration and expiration Constriction Doppler Inspiration Expiration Animation Animation Pitfalls and Caveats Subgroup of patients with constriction who do not exhibit respiratory changes COPD Constriction: Non-respirophasic Oh et. al. Circ. 1997;95:796-799 12 Pts. W/ confirmed constriction, but without the classic findings Deduced post Stripping, as Sx Not improve Etiology of Non-respirophasic pattern Mixed Restriction and Constriction Marked increase in Preload Preload reduction to unmask the respiratory variation Nasser S Tehrani:Wide STD.Deviation, But may be diagnostic for a ginven pt. Constriction: Non-respirophasic Supine Supine Insp. Sitting Expir. Sitting Insp. Expir. Effect of changing loading conditions w/ VALSALVA in RESTRICTION E 20% A to a lesser degree Pitfalls and Caveats Subgroup of patients with constriction who do not exhibit respiratory changes COPD COPD v.s. Constriction 100% change in E Velocity flow velocity profiles are not restrictive as LV filling Individual Mitral pressure is not increased. COPD v.s. Constriction COPD COPD: Greater than NL decrease in intrathroracic pressure is generated with inspiration => Increased SVC Flow Const. Constriction: Minimal change in SVC velocities with inspiration. Tissue Dopplar PW Analysis of Mitral Annular Motion Physiologic Premise: Assessment of VELOCITY of LV -Contraction, and -Relaxation How to: Apical 4 chamber minimizes the translational and rotational components of LV Contraction. TDI function of the machine is activated Gain is lowered to approx. Zero Wall filters are minimized to display lower velocities (annular velocity < 20 cm/s) Sweep at 100 or 200 mm/sec Tissue Dopplar: Restriction and Constriction Mitral inflow E wave is elevated in both Annular E wave Restriction, peak E-wave < 8 cm/sec Constriction, Peak E-wave > 8 cm/sec The above is Premised on the assumption that: Annular E wave is preload independent. Both Pro- and Con- studies regarding this premise exist. Constriction v.s. Restriction Dx has important therapeutic implications Clinical Presentaion similar: RHF Historical etiologies helpful, but not diagnostic A thick pericardium is not necessarily constrictive A restrictive process may constrict Echo and Hemodynamic features may overlap Restrictive Cardiomyopathy Broad categories of diseases involved: Etiologies of Constriction Infectious: Post-viral, TB, Purulent Traumatic:Post CABG, Pacer, Sternal trauma Post XRT Chronic inflammatory Dz: RA, SLE Uremia Neoplasia The End