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

• InfiltrativeAmyloidosisGaucher’sHunter’s, Hurler’s

• Storage diseaseHemochromatosisPompey (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 DiastoleCompliance

Atrial filling at end of Diastole

Parietal Parietal PericardiumPericardium Visceral Visceral

PericardiumPericardium

Pericardial Pericardial SpaceSpace

VENTRICULAR FILLING PHYSIOLOGY

CP

Early Diastole End DiastoleMid-Diastole

Normal Compliance Abrupt cessation of ventricular filling

-Fixed intracardiac volume-Ventricular Coupling-Pressure dissociation

CATH

LV

RV

D

D D D D D D D

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:

Constrictive Restrictive Sensitivity Specificity PPV NPV

EDP equalisation LVEDP-RVEDP < 5 mmHg LVEDP-RVEDP > 5 mmHg

EDP equalisation 60 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 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.

Hurrell et al. n=19p<0.05

Respiratory Dynamic Criteria

Cardiac Tamponade Physiology

Sharp et al. - 1960

INTRAPERICARDIALPRESSURE

INTRATHORACIC PRESSURE

PULMONARYWEDGE

PRESSURE

i e

NORMAL

““E.F.G.”E.F.G.”

““E.F.G.” E.F.G.” = Estimated Filling Gradient = Estimated Filling Gradient

Tamponade

““E.F.G.” E.F.G.” = Estimated Filling Gradient = Estimated Filling Gradient

PULMONARYWEDGE

PRESSURE

““E.F.G.”E.F.G.”

INTRAPERICARDIALPRESSURE

INTRATHORACIC PRESSURE

i e

Hatle et al, 1989

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

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=36 n=15

p<0.05

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

Respiratory changes in LVSP and RVSP

Hatle et al, 1989

Thank you …