Download Restrictive Cardiomyopathy

제 5회
회 한국 심초음파학회 Workshop
Restrictive Cardiomyopathy
Kye Hun Kim
The Heart Center of Chonnam National University Hospital
Cardiomyopathy: Historical Context
▶ Chronic myocarditis
: The only recognized cause of heart muscle disease in the mid
1850s
▶ Designation of primary myocardial disease
: Introduced in 1900
▶ Cardiomyopathy (CMP)
: Used for the first time in 1957
▶ WHO definition of CMP in 1968
: Diseases of different and often unknown etiology in which the
dominant feature is cardiomegaly and heart failure
Cardiomyopathy: Historical Context
▶ 1980 WHO classification of CMP
: Defined only as heart muscle diseases of unknown cause
: Reflecting general lack of information about causes or mechanism
▶ 1995 WHO definition of CMP
: Disease of myocardium associated with cardiac dysfunction
: Included newly recognized ARVD/C and primary restrictive CMP
for the first time
Classification of CMP
▶ WHO classification
▶ Functional classification
: Dilated, hypertrophic, restrictive
▶ Etiologic classification
▶ EMB histology classification
: Inflammatory/immune, infectious, infiltrative, cardiac tumors,
miscellanous specific (e.g., anthracycline), nonspecific
▶ Therapeutic classification
Classification of CMP
WHO Classification (1980)
WHO Classification (1995)
Classification of CMP
Functional Classification
Therapeutic Classification
Cardiomyopathy: WHO Classification (1995)
Normal
HCM
HCM
DCM
ARVD
RCM
EMF (OCM)
Needs for New Classification of CMP
▶ Identifications of several new disease entities
▶ Dramatic advances in diagnosis
▶ Precise knowledge of causation
▶ Rapid evolution of molecular genetics in cardiology
▶ Emergences of ion channelopathies, predisposing to
potentially lethal ventricular arrhythmias
New Definitions of CMP
▶ Major differences from 1995 WHO classification
: Genomic and molecular orientation of this proposed CMP
classification
▶ Classification according to contemporary molecular biology
: Not yet completely developed and thus probably premature
: Inadvisable at this time to preferentially formulate a classification
that is entirely dependent on genomics
New Definitions of CMP
▶ Not including as cardiomyopathies
: Valvular heart disease, systemic hypertension, congenital heart
disease, atherosclerotic coronary artery disease producing
ischemic myocardial damage secondary to impairment in
coronary flow (ICMP)
: Metastatic and primary intracavitary or intramyocardial cardiac
tumors, diseases affecting endocardium with little or no
myocardial involvement, and the imprecisely defined entity of
hypertensive HCM
New Classifications of CMP
▶ Two major groups
: Based on predominant organ involvement
▶ Primary CMP
: Genetic, mixed (genetic and nongenetic), acquired
: Solely or predominantly confined to heart muscle
▶ Secondary CMP
: Pathological myocardial involvement as part of a large number
and variety of generalized systemic disorders
Maron BJ et al. Circulation 2006; 113:1807-16
New Classifications: Primary CMP
PRIMARY CMP
(Predominantly involving the heart)
Genetic
PRKAG2
Danon
Mixed
HCM
DCM
Inflammatory
ARVC/D
Restrictive
(myocarditis)
LVNC
(non-hypertrophied
Stress-provoked
Glycogen
storage
and non-dilated)
(“Tako-Tsubo”)
Peripartum
Conduction
Defects
Tachycardiainduced
Mitochondrial
myopathies
Infants of insulindependent diabetic
mothers
Ion Channel
Disorders
PRKAG2
Acquired
Brugada
SQTS
CVPT
Asian SUNDS
Maron BJ et al. Circulation 2006; 113:1807-16
Restrictive CMP: Definition
▶ Rarely encountered forms of heart muscle diseases
▶ Characterized by diastolic heart failure
: Normal or decreased volume of both ventricles
: Biatrial enlargement, normal AV valves
: Impaired ventricular filling with restrictive physiology
: Usually normal systolic function
▶ Classified according to etiology as primary or secondary
Restrictive CMP: Etiology
▶ Primary RCMP (sporadic or familial)
▶ Secondary RCMP
: Amyloidosis
: Sarcoidosis
: Hemochromatosis
: Chemotherapy or radiation
: Hypereosinophilic syndrome
: Endomyocardial fibrosis
: Long-term chloroquine therapy
Familial Type RCMP
▶ Familial RCMP not related to amyloidosis are exceedingly rare
▶ Can be transmitted as an autosomal dominant trait
▶ Symptomatic disease that develops after the third decade of life,
with an insidious downhill course
▶ May be part of the spectrum of familial HCM in which there is
different phenotypic expression of the same genetic disease
: Mutation of troponin I was reported in some cases
Clinical Presentation
▶ Can present at any age, but its incidence is increased in the elderly
▶ More common in older women than men
▶ Signs of both pulmonary and systemic congestion
: Dyspnea, peripheral edema, palpitations, fatigue, weakness, and
exercise intolerance
: In advanced cases, hepatosplenomegaly, ascites, and anasarca
Cardiovascular Examination
▶ Often indistinguishable from that of constrictive pericarditis
▶ Elevated jugular venous pressure with prominent y descent
▶ An inspiratory increase in venous pressure (Kussmaul's sign)
▶ Left ventricular impulse
: Usually normal
: Non-palpable impulse suggestive of constrictive pericarditis
▶ S1 and S2 are usually normal, S3 gallop is frequently present
▶ Soft systolic murmurs of functional MR and TR
Case: 김 O O (68/M)
CC
DOE (NYHA III/IV) and leg edema
VS
BP 110/ 70 mmHg
BT 36 ℃
PR 96 /min
RR 20 /min
PE
Pansystolic murmur
Jugular vein engorgement
Decreased BS on both lower lung fields
Pitting edema on both legs
김 O O (68/M): Electrocardiography
김 O O (68/M): Chest X-ray
Diagnostic Evaluation
Heart failure by history and examination
Echocardiography
LVEF ≥ 50%
Rule out aortic stenosis, HT,
hypertrophic CMP
Doppler echocardiography
LVEF < 40%
Non-dilated LV
DCMP
Work-up for DCMP
Diagnostic Evaluation
Doppler echocardiography
:
:
:
:
:
:
:
:
Increased early diastolic filling velocity (E)
Decreased atrial filling velocity (A)
E/A ≥ 1.5
Decreased deceleration time
Decreased isovolumic relaxation time
Markedly decrease in the ratio of PVs/PVd
Augmented atrial reversal velocity
Markedly reduced E` and A`
Probable RCMP
Inconclusive
CT scan or MRI: pericardial thickening
Probable CP
Inconclusive
Cardiac catheterization
Diagnostic Evaluation
Cardiac catheterization
: Elevated RA pressure with prominent x and y descent
: Classic square-root sign
: RV systolic pressure ≥ 50mmHg
Probable RCMP
: RV diastolic pressure less than 1/3 of RVSP
: LVEDP is typically greater than RVEDP by 5mmHg or more
: Separation of LVEDP from RVEDP with volume challenge if
equalization present
: Absence of discordance in RVSP and LVSP during respiration
Inconclusive
CP or Primary RCMP
EMB: diagnostic of specific RCMP
Inconclusive
김 O O (68/M): Echocardiography
김 O O (68/M): Echocardiography
김 O O (68/M): Echocardiography
김 O O (68/M): Echocardiography
김 O O (68/M): Echocardiography
Diagnostic Evaluation: Echocardiography
▶ Shortened DT: < 160ms
▶ Shortened IVRT: < 70ms
▶ E/A: > 1.5 - 2.0
▶ PVs2 << PVd
▶ Increased Pva velocity: > 35cm/s
▶ Vp < 45 cm/s
▶ Mitral A duration < Pva duration
▶ E/E` > 15
Diagnostic Evaluation: Echocardiography
Restriction versus Constriction
▶ RCMP and CP have similar physiology
: Differentiating two conditions may be difficult
▶ Hepatic venous flow
: Reversal of forward flow during expiration in constriction, since
the RV becomes less compliant as the LV fills more
: Usually increased reversal flow during inspiration in restriction
▶ Color M-mode
: Vp < 45cm/s in restriction, Vp ≥ 45 cm/s in constriction
Restriction versus Constriction
▶ Early diastolic Doppler tissue velocity at the mitral annulus (E')
: Decreased in restrictive CMP due to an intrinsic decrease in
myocardial contraction and relaxation
: Increased in constrictive pericarditis, since the longitudinal
movement of the myocardium is enhanced
▶ High E' velocity (>12 cm/sec) indicates constrictive pericarditis
▶ Low E' velocity (<8 cm/sec) indicates restrictive CMP
▶ Ventricular interdependence between mitral and tricuspid inflow
: Reciprocal change between mitral and tricuspid inflow according
to the respiration in constriction, but not in restriction
Restriction versus Constriction
Restriction versus Constriction: Coronary Blood Flow
▶ Reductions in coronary flow reserve and peak hyperemic flow
velocity in either constrictive pericarditis or RCMP
▶ Velocity half time of diastolic blood flow
: Velocity half-time < 380 msec predicts the presence of either
constrictive pericarditis or RCMP versus normal controls with
high sensitivity (100%)and specificity (100%).
: Velocity half-time < 260 msec or that corrected by sq rt RR <9.5
of diastolic blood flow distinguished constrictive pericarditis
from restrictive cardiomyopathy with a sensitivity and specificity
of 86 and 88 percent
Akasaka T et al. Circulation 1997; 96:1874
Restriction versus Constriction: Coronary Blood Flow
Akasaka T et al. Circulation 1997; 96:1874
Diagnostic Evaluation: Endomyocardial Biopsy
▶ Performed in patients with suspected RCMP
▶ To exclude specific heart muscle disease such as amyloidosis,
sarcoidosis, and hemochromatosis
▶ Highly specific for excluding specific heart muscle diseases and
myocarditis
▶ LM examination
: Nonspecific patchy endocardial and interstitial fibrosis with
increased collagen deposition, myocellular hypertrophy without
any myofiber necrosis or disarray, and without lymphocytic or
eosinophilic infiltration, or amyloid or iron deposition
Treatment
▶ No specific therapy for idiopathic restrictive cardiomyopathy
▶ Therapy of certain underlying diseases
▶ Aimed at reducing pulmonary and systemic congestion
: Lowering the venous pressure
: Controlling heart rate
: Increasing filling time
: Maintenance of atrial contractions
: Correction of atrioventricular conduction disturbances
: Avoidance of anemia, nutritional deficiency, calcium overload,
and electrolyte imbalance.
Treatment
▶ Loop diuretics
: Usually in low to medium doses
: BUN and serum Cr concentration should be carefully monitored
▶ Rate-lowering calcium channel blockers (eg, verapamil)
: Improving diastolic function via rate control and increasing
ventricular filling time
▶ Beta blockers
: By suppressing the long-term deleterious consequences of
compensatory sympathetic stimulation on myocyte function, by
controlling the heart rate (which increases filling time), and by
improving ventricular relaxation
Treatment
▶ ACE inhibitors and/or ARB
: Improve diastolic filling by counteracting the compensatory
neurohormonal changes associated with heart failure
▶ Permanent dual chamber pacemaker
: In the presence of advanced atrioventricular block
▶ Cardiac transplantation
: In patients with intractable heart failure
Prognosis
▶ Poor Prognosis
: Advanced diastolic dysfunction (grade III to IV) in secondary
restrictive cardiomyopathy such as cardiac amyloidosis.
: Symptomatic idiopathic restrictive cardiomyopathy
▶ Adverse risk factors for survival
: Male gender
: Age greater than 70
: Increment in functional class
: Left atrial diameter >60 mm