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 of: Traditional Hemodynamic Criteria are of LIMITED Utility 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: Therefore, to understand Restriction, must start with a discussion of Diastole 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 incresase Systlic failure as the only kind of failure recongized LV-EDV => diastole more than just passive LV as an active suction 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: IVRT>100 ms Earliest diastolic ab-normality. Impaired LV relax. Filling pressures=NL Dz. progression: Decreased LV compliance, and Increased filling pressure More specific quantitative approach to classification of Diastole Late diastolic filling Diastasis Early diastolic filling IVRT

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 Tachycardia PR prologgation “A” wave duration……. Utilized in relation to PV “a” wave duration.

E:A ratios, as a Function of Age Impaired LV relaxation (IVRT ) Decreased LV Compliance COPIED LATER Restrictive Pseudonormal

The three abnormal LV filling patterns Comment on how do you know if it is pseudo, when no baseline patterns are available ala ECGs? ANSWER: Compare with the PV flow pattern//// Cut out the last row, and save it and the next slide for a juxaposition of PV in RESTRICTION vs CONSTRICTION ala KLEIN’s paper.. COPIED LATER

Pathophysiologic Similarity of: Restriction and Constriction Abnormal increase in ventricular pressure impeding filling of the LV To NL EDV Constriction Restriction Myocardial Disorder 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. Visiting at MAYO from Holland at the time. 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 Ventricular Pressures Are DISCORDANT Insp Expir

Traditional v.s. Dynamic Catheterization Hemodynamics Why bother with Echo given The great utility of Dynamic Respiratory cath measurments? These measurments are only Possible using High-fidelity Micromanometer systems (not a common practice). Dissociation of Intrathoracic and Intracardiac Pressures

Effect of Inspiration: Constriction Expir. Insp. Expir. PCWP PCWP Inspir. Expir. PCWP No proportionate decrease in LV diastolic pressure Decreased transmitral gradient => Transmitral flow RV SV LV SV

Pathophysiologic Differences Constriction Restriction Ab-Nl Myocardial compliance Myocardial compliance is NL No impedence to Diastolic EARLY FILLING Impedence to filling increases throughout the diastole Total cardiac volume is fixed by the pericardium Pericardium is compliant Septum is non-compliant Reduction of the proportion of LV filling with atrial contraction: => Atrial enlargement Atria are able to empty into the Ventricles, though at higher Press. Minimal Respiratory effect of RV on the LV Marked Respiratory effect of LV on the RV

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: There is no respiratory variation of Mitral inflow

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 RV-SV, w/ LA pressure and compliance. PVa duration- Interplay of multiple factors Now go back to the previous Slide and drive home the point regarding the Dx of Pseudo Utilized in relation to Mitral “A” wave duration.

Three abnormal PV filling patterns in Restriction Seen this slide once before

E:A ratios, as a function of Age Impaired LV relaxation (IVRT ) Decreased LV Compliance Seen this slide once before 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 Above findings suggestive of elevated LV end diastolic pressure 121 ms

PVs are best assessed using TEE PV interrogation using TTE is often techniaclly limited. PVs are best assessed using TEE

Normal PV Flow-TEE NO Variation from Inspiation to Expiration Rt. Upper PV NO Variation from Inspiation to Expiration Kline-1937 LV inflow

PV Dopplar Patterns in Restriction-TEE Lt. Upper PV PV flow is not respirophasic Systolic/Diastolic velocity is markedly down in both inspiration and expiration Kline 1939, 1993JACC LV Inflow LV inflow Peak-E velocity is not respirophasic

PV Dopplar Patterns in Costriction-TEE Lt. Upper PV PV flow 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 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 Systolic and diastolic forward flow S-vel. > D-vel. Diastolic flow reversal: Expir.>>Insp.

Hepatic Vein Dopplar: Constriction Diastolic flow reversal is augmented in expiration. DFRexp.>25% forward diastolic velocity

Hepatic Vein Dopplar: Restriction Forward flow primarily in Diastole. Inspiration increases both >systolic, and >Diastolic Flow reversals.

Hepatic Vein Dopplar: Compilation 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 Such Pts comprised 12% of Pts in a series of ???, reported by Oh JACC 1994;23:154-162

Constriction: Non-respirophasic Oh et. al. Circ. 1997;95:796-799 12 Pts. W/ confirmed constriction, but without the classic findings Etiology of Non-respirophasic pattern Mixed Restriction and Constriction Marked increase in Preload Oh Circ 1997; 95:796-799 Deduced post Stripping, as Sx Not improve Preload reduction to unmask the respiratory variation

Constriction: Non-respirophasic Nasser S Tehrani:Wide STD.Deviation, But may be diagnostic for a ginven pt. Constriction: Non-respirophasic Supine Supine Sitting Oh 180-A Insp. Expir. Insp. Expir. Sitting

Effect of changing loading conditions w/ VALSALVA in RESTRICTION 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 Individual Mitral flow velocity profiles are not restrictive as LV filling pressure is not increased.

COPD v.s. Constriction COPD: Greater than NL Increased SVC Flow COPD 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: 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 Apical 4 chamber minimizes the translational and rotational components of LV Contraction. THEREFORE, ONLY AXIAL COMPONENT OF VELOCITY IS INTACT

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