Cardiomyopathy.pptx2!5!13 3 Year Mbbs

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DR ABDUL GHANI WASEEM



TYPES IDIOPATHIC DILATED CARDIOMYOPATHY



ETIOLOGIES OF DILATED CARDIOMYOPATHY

0

5

10

15

20

25

30

35

40

45

50

Disorder

IDCM

Myocarditis

Ischmic CM

InfiltrativediseasePeripartum CM

Hypertension

HIV

CTD

Substanceabuse



IDIOPATHIC DILATED CARDIOMYOPATHY

PATHOLOGY

Four chamber dilatation

Mild to moderate ventricular hypertrophy

Varying degrees of interstitial fibrosis and

myocyte hypertrophy

“Functional” atrioventricular regurgitation is

common



IDIOPATHIC DILATED CARDIOMYOPATHYPATHOLOGIC FINDINGS



IDIOPATHIC DILATED CARDIOMYOPATHY

PATHOGENESIS Familial/genetic factors

Viral myocarditis and cytotoxic insults

Immunologic abnormalities Beta-receptor auto-antibodies

Abnormal T-cell function

Metabolic, energetic, and contractile

abnormalities Ca2+-ATPase

Myofibrillar ATPase

Creatine Kinase



MOLECULAR DEFECTS IN DILATEDCARDIOMYOPATHY

GENESLamin A/C

δ-sarcoglycan

Dystrophin

Desmin

Vinculin

Titin

Troponin-T

α-tropomyosin

ß-myosin heavy chain

Actin

Mitochondrial DNAmutations



FAMILIAL DILATED CARDIOMYOPATHYCOMMON ASSOCIATED ABNORMALITIES

Conduction system disease

Skeletal muscle myopathy or muscular dystrophy

X-linked and autosomal dominant inheritancepatterns are most common

Extracardiac manifestations:

Sensorineural hearing loss Neutropenia



NON-INVASIVE EVALUATION OF MYOCARDITIS MRI IMAGING

Unenhanced Enhanced



IDIOPATHIC DILATED CARDIOMYPATHY

EPIDEMIOLOGY

ANNUAL INCIDENCE 5-8/100,000

PREVELANCE 36/ 100,000

INCREASED RISK ASSOCIATED WITH: MALE GENDER

BLACK RACE

HYPERTENSION

CHRONIC BETA-AGONIST USE



IDIOPATHIC DILATED CARDIOMYPATHY

CLINICAL PRESENTATIONS

Heart failure symptoms 75%-85%

Anginal chest pain 8%-20%

Emboli (systemic or pulmonary) 1%-4%

Syncope <1%

Sudden cardiac death <1%



SPONTANEOUS IMPROVEMENT IN ACUTE

DILATED CARDIOMYOPATHY

PATIENT POPULATION

49 patients with heart failure symptoms of lessthan 6 months duration were compared to a

cohort of 248 chronic dilated cardiomyopathypatients

Improvement was prospectively defined as a rise

in LVEF > 0.15 to a final value of > 0.30

-Steimle AE et al. JACC 1994;23:553-9

ACUTE DILATED CARDIOMYOPATHY



ACUTE DILATED CARDIOMYOPATHYOUTCOME

49 Patients with Recent Onset Cardiomyopathy

12 Died/10 Tx 16 Alive & Unimproved 11 Improved

18 Died/13 Tx 5 Alive & Unimproved 13 Improved

11±15 mos 27 ± 22 mos 43 ± 29 mos

12 months

Steimle et al JACC 1994;23:553-9

2 9 5

9



SPONTANEOUS IMPROVEMENT IN ACUTE

DILATED CARDIOMYOPATHY

UNIVARIATE PREDICTORS OF IMPROVEMENTshort duration of symptomshigher cardiac outputlower NYHA functional classificationsmaller LV end-diastolic dimensionlower filling pressures

higher serum sodium concentration

STEPWISE REGRESSION MODELshort duration of symptomshigher serum sodium concentrationlower right atrial pressure

lower pulmonary capillary wedge pressure

IDCM PROGNOSTIC FEATURES



IDCM:PROGNOSTIC FEATURES

VENTRICULOGRAPHIC FINDINGS

Degree of impairment in LVEF Extent of left ventricular enlargement

Coexistent right ventricular dysfunction

Ventricular mass/volume ratio

Global wall motion abnormalities

Left ventricular sphericity

CLINICAL FINDINGS

Favorable prognosis: NYHA < IV, younger age, femalesex

Poor prognosis: Syncope, persistent S3 gallop, right-sided heart failure, AV or bundle branch block,hyponatremia, troponin elevation, increased BNP,maximum oxygen uptake < 12 mg/kg/min



ACC/AHA HEART FAILURE EVALUATION GUIDELINES

CLASS I & II RECOMMENDATIONS

Laboratory Studies

Blood count, urinalysis, electrolytes, renal function,glucose, LFTs (class I; level C)

Thyroid stimulating hormone (class I; level C)

Fe/TIBC, ferritin (class IIa, level C)

Urinary screening for hemochromatosis (class IIa; level C) Measurement of ANA, rheumatoid factor, urinary VMA and

metanepherines in selected patients (class IIa; level C)

HIV testing (class IIb; level C)

Electrocardiogram (class I; level C)

Chest x-ray (class I; level C)



DILATED CARDIOMYOPATHYELECTROCARDIOGRAPHIC FINDINGS

Disease Etiology Pathologic Q-waves

Ischemic cardiomyopathy 10/12 (83%)*

(n=15)

Idiopathic cardiomyopathy 2/21 (10%)+ #

(n=21)

*LBBB (n=2); paced rhythm (n=1)

+ LVH (n=10);

IVCD (n=3)# P < 0.003



SEGMENTAL WALL MOTION ABNORMALITIES INDILATED CARDIOMYOPATHY

Regional wall motion abnormalities observed in at least50% of patients with non-ischemic causes of dilatedcardiomyopathy

Most frequent wall motion abnormalities: anterior wall & apex

Posterior and lateral walls most likely to be preserved

Type of abnormality: hypokinesis (83%) akinesis (11%)

dyskinesis (6%)

Heterogeneity in regional oxidative metabolism using C-11acetate clearance has been demonstrated in DCM



NONINVASIVE ASSESSMENT OF CORONARY ARTERY

DISEASE IN NEW ONSET DILATED CARDIOMYOPATHY

Retrospective studies have shown up to 94% of patientswith idiopathic dilated cardiomyopathy will havemyocardial perfusion defects Reversible defect(s): 60%

Fixed defect(s): 15%

Reversible+ fixed defect(s): 25%

Global myocardial blood flow reserve (dipyridamole-induced) is diminished in DCM patients compared tocontrols using PET imaging

Low myocardial blood flow reserve correlates with highleft ventricular wall stress and anaerobic metabolism

Ann Inter Med 1992;152:679-72; JACC 2000;35:19-28.



INDICATIONS FOR CORONARY ANGIOGRAPHY IN NEW

ONSET CARDIOMYOPATHYACC/AHA CONSENSUS GUIDELINES (2001)

Patients with Known Coronary Artery Disease/Angina Pectoris

Revascularization recommended in vast majority of such individuals

with multivessel disease. Little role for non-invasive testing.

Coronary angiography considered Class I Recommendation (Level of

evidence: B)

Patients with Known Coronary Artery Disease Who Lack Angina

No controlled trials have examined whether coronary revascularization

can improve outcomes in this population

Many centers first evaluate patient for myocardial hibernation

Coronary angiography considered Class IIa Recommendation (Level of

Evidence:C)

Patients with or without Chest Pain in Whom Coronary Artery

Disease has Not Been Evaluated

Approximately 35% of patients with IDCM will report angina-like pain

Coronary angiography should be considered Class IIa recommendation

(Level of Evidence: C)

Hunt SA,et al. Circulation 2001;104:2996

ENDOMYOCARDIAL BIOPSY IN DILATED CARDIOMYOPATHY



RIGHT

VENTRICULAR

BIOPSY

TECHNIQUE

ENDOMYOCARDIAL BIOPSY IN DILATED CARDIOMYOPATHY



INDICATIONS FOR ENDOMYOCARDIALBIOPSY

Acute dilated cardiomyopathy with refractory heart failuresymptoms

Rapidly progressive ventricular dysfunction in an unexplainedcardiomyopathy of recent onset

New onset cardiomyopathy with recurrent ventricular tachycardia or high grade heart block

Heart failure in the setting of fever, rash, and peripheraleosinophilia

Dilated cardiomyopathy in setting of systemic diseases

known to affect the myocardium (systemic lupus erythematosus,polymyositis, sarcoidosis)

Wu LA, et al. Mayo Clin Proc 2001;76:1030-8



SURVIVAL BY HISTOPATHOLOGICAL TYPE OFMYOCARDITIS

CP977755-6

0.0

0.2

0.4

0.6

0.8

1.0

0 1 2 3 4 5

Survival (yr)

P

r o p o r t i o n s

u r v i v i n g

GCM group

LM group

Cooper, et al NEJM 1997



DILATED CARDIOMYOPATHY PROVEN THERAPEUTIC OPTIONS

TREATMENT INDICATIONS

ACE Inhibitors Symptomatic heart failure andasymptomatic LV dysfunction

ARBs ACE intolerance

Hydralazine- nitrates ACE intolerance

Diuretics Volume overloadPotassium/Magnesium Diuretic-induced depletion

Beta-blockers Symptomatic heart failure in addition to ACE inhibitor

Digoxin Persistent heart failure despitediuretics, ACE inhibitor

Warfarin Chronic or paroxysmal atrial fibrillation

LV thrombus or prior embolic event

ICD Cardiac arrest; uncontrolled VT



STATIN THERAPY IMPROVES VENTRICULAR

FUNCTION IN DILATED CARDIOMYOPATHY

Node K, et al. Circulation 2003;108:839-43



CONTROLLED TRIAL OF IMMUNE GLOBULIN IN

RECENT ONSET DILATED CARDIOMYOPATHY

Purpose: To determine whether intravenous immunoglobulin G

(IVIG) improves ejection fraction in adults with recent onset

idiopathic dilated cardiomyopathy or myocarditis

Methods: 62 patients with symptomatic DCM < 6 months and

LVEF < 40% were randomized to receive IVIG 2 g/kg or placebo

Study Population:

Age (mean) 43 ± 12 yrs

LVEF 25 ± 8%

Symptom duration 2.0 ± 1.5 months

Myocarditis 16%

McNamara et al. Circulation 2001;103:2254-9



IMMUNOGLOBULIN THERAPY FOR ACUTE DILATEDCARDIOMYOPATHY:IMAC TRIAL RESULTS

McNamara et al. Circulation 2001;103:2254-9

IMMUNOADSORPTION THERAPY FOR DILATED




CARDIOMYOPATHY

12 MONTH AUTOANTIBODY LEVELS BY TREATMENT

GROUP

Muller J et al. Circulation 2000;101: 385 - 391




IMMUNOADSORPTION THERAPY FOR DILATEDCARDIOMYOPATHY12 MONTH CHANGE IN EJECTION FRACTION BY TREATMENT

GROUP

Muller J et al. Circulation 2000;101: 385 - 391



EFFECT OF REMOVAL OF ANTIBODIES BYIMMUNOADSORPTION IN DILATED CARDIOMYOPATHY

Felix SB, et al. JACC 2002;39:646-52

n=12

Effect of

column

effluent on

adult rat

cardiocyte

contractility

CONTROLLED TRIAL OF IMMUNOADSORPTION AND




IMMUNOGLOBULIN SUBSTITUTION IN DILATEDCARDIOMYOPATHY

Hypothesis: Immunomodulatory therapy may decreasemyocardial inflammation and improve ventricular systolicfunction

Methods: 25 patients with DCM were randomized toimmunoabsorption (IA) followed by IgG (0.5 gm/kg)

replacement for 3 consecutive months (n=12) or conventional therapy (n=13):

Age: 50 ± 11 years

LVEF: 20% ± 6%

Symptom Duration: 4.0 yearsFibrosis: 8.7%

Primary End-points: Change in LVEF (3 month)

Change in CD3+, CD4+ & CD8+ cells

Staudt A et al. Circulation 2001;103:2681-8



IMMUNOABSORPTION AND REPLACEMENT

TREATMENT FOR DILATED CARDIOMYOPATHYCHANGES IN CELLULAR INFILTRATION (3 mon ths )


IA/IgG treatment

resulted in a

significant declinein all subtypes of

infiltrating

lymphocytes

** p < 0.05 vs baseline

++ p < 0.05 vs controls



IMMUNOABSORPTION AND REPLACEMENTTREATMENT FOR DILATED CARDIOMYOPATHY


A marked

decrease inmyocardial HLA-

class II antigen

expression is

evident after 3

months of

treatment

(magnification X

400)




IMMUNOGLOBULIN SUBSTITUTION IN DILATED CARDIOMYOPATHY

CHANGE IN LEFT VENTRICULAR FUNCTION (3 Months)

**p <0.05 vs baseline

++p < 0.01vs controls




IMMUNOSUPPRESSIVE THERAPY FORINFLAMMATORY DILATED CARDIOMYOPATHY

Purpose: To assess the efficacy of immunosuppressive therapy in patients

with dilated cardiomyopathy and HLA up-regulation on biopsy.

Study Population: 84 (of 202 DCM) patients had HLA class I or II

expression on myocytes, endothelium or interstitial cells and were

randomized to 24 months of conventional therapy [ digoxin, furosemide,spironolactone, ACE inhibitor, beta-blocker, nitrates, and amiodarone]

alone or with concomitant immunosuppression [ prednisone 1mg/kg/day

taper to 0.2 mg/kg/day for 90 days + azathioprine 1 mg/kg/day for 100

days].

Primary Endpoint: Death, transplantation or hospital readmission

Secondary Endpoints: LVEF, LVEDD, LVESD, NYHA class

Wojnicz R, et al. Circulation 2001;104:39-45

IMMUNOSUPPRESSIVE THERAPY FOR DILATED



IMMUNOSUPPRESSIVE THERAPY FOR DILATED

CARDIOMYOPATHYCHANGE IN VENTRICULAR FUNCTION

0%

5%10%

15%

20%

25%

30%

35%

40%

45%

Baseline 3 Month 6 Month 12 Month 24 Month

Placebo

Immuno

Left Ventricular Ejection Fraction

Wojnicz R, et al. Circulation 2001;104:39-45



ALIAN UNCONTROLLED IMMUNOSUPPRESSIVE TRIAL

R MYOCARDITIS

112 patients had biopsy-proven lymphocytic myocarditis41 patients had progressive symptoms for > 3 months

duration and were treated with 6 months with

prednisone (1 mg/kg/day x 4 wks; 0.33 mg/kg/day x 5 months)

and azathioprine (2 mg/kg/day x 6 months)

Efficacy of therapy was evaluated at 6 & 12 months

Responders demonstrated:Decrease in NYHA class

Increase in LVEF > 10 Units

Frustaci A, et al. Circulation 2003;107:857-63



ITALIAN UNCONTROLLED TRIAL OF IMMUNOSUPPRESSIVE

THERAPY FOR MYOCARDITIS

0.00%

10.00%

20.00%

30.00%

40.00%

50.00%

LVEF

1 2 3


BASELINE 6 MO 12 MO

RRR



IMMUNOSUPPRESSIVE THERAPY FOR MYOCARDITIS

STUDY DESIGN

RESPONDERS NON-RESPONDERS(N=21) (N=20)


Viral Genome 3 (14%) * 17 (85%) +

Cardiac Antibodies 19 (90%)# 0 (0%)

* P < 0.001; # p < 0.001

+ Enterovirus 5; EB virus 5; adenovirus 4; influenza

1; parvovirus 1



TREATMENT FOR IDIOPATHIC DILATED

CARDIOMYOPATHY

2004 AND BEYOND

Conventional neurohormonal antagonists

? Anticoagulation (WATCH; WARCEF)

? ICD implantation (DEFINITE & SCD-HeFT)

? Immunosuppression vs immunomodulation

Gene therapy (SERCA2a, phospholamban)

Cellular transplantation

Fetal cardiomyocytes Skeletal myoblasts

Adult (tissue) stem cells

Embryonic stem cells

IMAC TRIAL RESULT:APOPTOSIS AND



668N =

Fas gene expression

HighModerateLow

50.0

40.0

30.0

20.0

10.0

0.0

-10.0

-20.0

668N =

Fas gene expression

HighModerateLow

40.0

30.0

20.0

10.0

0.0

-10.0

Fas Expression and LV Recovery

p=0.002 p=0.006

Six months Twelve months

Sheppard, AHA 2003

IMAC TRIAL RESULT:APOPTOSIS AND

RECOVERY OF VENTRICULAR FUNCTION



Hypertrophic Cardiomyopathy



Definition WHO

Left and/or Right ventricular hypertrophy, usually asymmetric and involves the interventricular septum.



Differential Diagnosis:

HCM

Can be asymmetric

Wall thickness: > 15 mm

LA: > 40 mm

LVEDD : < 45 mm

Diastolic function: always

abnormal

Athletic heart

Concentric & regresses

< 15 mm

< 40 mm

> 45 mm

Normal



Stimulus Unknown

Disorder of intracellular calcium metabolism

Neural crest disorder

Papillary muscle malpositioned and misoriented



Genetic abnormality Autosomal dominant.

Mutations in genes for cardiac sarcomeric proteins.

Polymorphism of ACE gene.

ß-myosin heavy chain gene on chromosome 14.



Variants of HCM

Most common location: subaortic , septal, and ant. wall.

Asymmetric hypertrophy (septum and ant. wall): 70 %.

Basal septal hypertrophy: 15- 20 %.

Concentric LVH: 8-10 %.

Apical or lateral wall: < 2 % (25 % in Japan/Asia):characteristic giant T-wave inversion laterally & spade-like left ventricular cavity: more benign.



Hypertensive hypertrophic Cardiomyopathy

Elderly women

Simulates HCM

Prognosis better than non-hypertensive HCM



Pathophysiology of HCM Dynamic LV outflow tract obstruction

Diastolic dysfunction

Myocardial ischemia Mitral regurgitation

Arrhythmias



Left ventricular outflow tract gradient

↑ with decreased preload, decreased afterload, orincreased contractility.

Venturi effect: anterior mitral valve leaflets & chordaesucked into outflow tract→

↑ obstruction, eccentric jet of MR in mid-late systole.



Maneuvers that ↓ end-diastolic volume

(↓ venous return & afterload, ↑ contractility)

Vasodilators

Inotropes

Dehydration

Valsalva

Amyl nitrite Exercise

→ ↑HCM murmur



Arrhythmias:

Sustained V-Tach and V-Fib: most likely mechanism of syncope/ sudden death.

Dependant on atrial kick: CO ↓ by 40 % if A. Fibpresent.



Histology: Myocardial fiber disarray, endocardial plaques.

Abnormal relaxation and diversely orientedmyocardial fibers.

Intimal hyperplasia of intramural coronary arteries,endothelial dysfunction, myocardial perfusion defects.



Clinical presentation: Any age

Leading cause of sudden death in competitive athletes

Triad: DOE, angina, presyncope/syncope.



Physical exam: Apex localized, sustained

Palpable S4

Tripple ripple

Prominent “a” wave

Rapid upstroke carotid pulse, “jerky” bifid (spike-and-dome pulse)

Harsh systolic ejection murmur across entireprecordium→ apex & heart base

MR: separate murmur: severity of MR related todegree of outf low obstruction



EKG:



Echocardiography:

2D-echo:

Asymmetric septal hypertrophy

Diffuse concentric or localized to apex/anterior wall

Systolic anterior motion of MV (SAM)



Doppler Echocardiocraphy:

Typical appearance: late-peaking signal “dagger-shaped”

Bernoulli for peak systolic gradient(+ maneuvers)

Obstructive or non-obstructive

Distinguish MR and intra-cavitary obstruction(looking for the aortic closure signal)



Cardiac cath:

Not necessary



Brockenbrough response ↑ LV systolic pressure

↓ Ao systolic pressure

↑ gradient between LV & Ao

Post PVC



Brockenbrough response



Imitator of HCM Amyloidosis:

Thickened walls & low voltage on EKG.



Natural history of HCM Mortality: 3 %/year (6-8 % with NSVTach)

Poor prognosis:

- Younger age- Male sex

- + family hx. of sudden death

- Hx. of syncope- Genetic markers (mutations of arginine gene)

- Exercise-induced hypotension (worst)



Genetic defect and prognosis



Management All first degree relatives: screening…

echocardiography/genetic counseling

Avoid competitive athletics

Prophylactic antibiotics before medical & dentalprocedures

Holter x 48 hours



β- Blockers: Propranolol 200-400 mg/d

(large doses)/ Selective β- B lose selectivity at highdoses:

Slow HR → longer diastolic filling time → ↓ myocardial O2 consumption →

↓ myocardial ischemia & LVOT obstruction

CaCh- Blockers: Verapamil 240-320 mg/d(with caution for hemodynamic deterioration)

Combination of both



Disopyramide: class I antiarrhythmic + strong –iveinotropic effect

Non-responders to Medical therapy



p py

???

1- Surgery (Myotomy/Myectomy) +/- MVR

2- ICD

3- DDD pacemaker

4- NSRT (alcohol septal ablation)



1- Surgery:Septal myotomy/myectomy:

Patients < 40 years: mortality < 1 %

Patients > 65 years: mortality 10-15 %

Survival better than medically treated patients Should be considered in: resting gradient > 50 mmHg, or

refractory to medical Rx.

Young patients, particularly those with severe disease

Additional structural abnormalities affecting the mitral valve or coronary arteries.

Complication (rare): Aortic incompetence



Myotomy/Myectomy



2- ICD: Previous sudden death

High risk of sudden death

EPS use ?



3- DDD pacemakerSubstantial ↓ gradient(~ 50 %)



Effect of DDD pacemaker in HCM



P t ti l M h i f b fit f P i i HCM



Potential Mechanisms of benefit of Pacing in HCM:

RV apical pacing & maintenance of AV synchrony → abnormal pattern of septal contraction → ↓ early systolic bulging of hypertrophic subaortic septum in

LVOT &↓ Venturi forces that produce SAM.

↑ LVOT width during systole

↓ systolic hypercontractility: ↑ end-systolic volume→

↓ intraventricular pressure gradients & myocardial work



↓ MR

May favorably alter diastolic function

LVH regression



Candidates for DDD



4- Alcohol septal ablation (NSRT)

Controlled myocardial infarction

of the basal ventricular septum

to ↓ gradient. First septal artery occluded with

a balloon catheter and ETOH

injected distally



NSRT (Non Surgical Septal Reduction Therapy)The most appropriate candidates for NSRT should meet all of thefollowing criteria :

- HCM with severe symptoms of heart failure (NYHA class III to IV)

despite adequate tolerated drug therapy

- An LVOT gradient 50 mmHg at rest or after exercise or >30 mmHg atrest or 60 mmHg under stress

- Basal septal thickness 18 mm

- NYHA class II heart failure with a resting LVOTgradient >50 mmHg or>30 mmHg at rest and 100 mmHg with stress .

- Elderly or comorbidities that may increase the risk of surgical

correction.



H hi C di h



Hypertrophic Cardiomyopathy



Definition:

WHO: left and/or right ventricular

hypertrophy, usually asymmetric and

involves the interventricular septum.



Differential Diagnosis:

HCM

Can be asymmetric

Wall thickness: > 15 mm

LA: > 40 mm

LVEDD : < 45 mm

Diastolic function: always

abnormal

Athletic heart

Concentric & regresses

< 15 mm

< 40 mm

> 45 mm

Normal



Stimulus:

Unknown

Disorder of intracellular calcium metabolism

Neural crest disorder

Papillary muscle malpositioned and misoriented



Genetic abnormality: Autosomal dominant.

Mutations in genes for cardiac sarcomeric proteins.

Polymorphism of ACE gene.

ß-myosin heavy chain gene on chromosome 14.



Variants of HCM:Most common location: subaortic , septal, and ant. wall.

Asymmetric hypertrophy (septum and ant. wall): 70 %.

Basal septal hypertrophy: 15- 20 %.

Concentric LVH: 8-10 %.

Apical or lateral wall: < 2 % (25 % in Japan/Asia):characteristic giant T-wave inversion laterally & spade-like left ventricular cavity: more benign.



Hypertensive hypertrophic Cardiomyopathy

Elderly women

Simulates HCM

Prognosis better than non-hypertensive HCM



Pathophysiology of HCM Dynamic LV outflow tract obstruction

Diastolic dysfunction

Myocardial ischemia

Mitral regurgitation

Arrhythmias



Left ventricular outflow tract gradient

↑ with decreased preload, decreased afterload, orincreased contractility.

Venturi effect: anterior mitral valve leaflets & chordaesucked into outflow tract→

↑ obstruction, eccentric jet of MR in mid-late systole.



Maneuvers that ↓ end-diastolic volume

(↓ venous return & afterload, ↑ contractility)

Vasodilators

Inotropes

Dehydration

Valsalva

Amyl nitrite Exercise

→ ↑HCM murmur



Arrhythmias:

Sustained V-Tach and V-Fib: most likely mechanism of syncope/ sudden death.

Dependant on atrial kick: CO ↓ by 40 % if A. Fibpresent.



Histology: Myocardial fiber disarray, endocardial plaques.

Abnormal relaxation and diversely orientedmyocardial fibers.

Intimal hyperplasia of intramural coronary arteries,endothelial dysfunction, myocardial perfusion defects.



Clinical presentation:

Any age

Leading cause of sudden death in competitive athletes

Triad: DOE, angina, presyncope/syncope.



Physical exam:

Apex localized, sustained

Palpable S4

Tripple ripple

Prominent “a” wave Rapid upstroke carotid pulse, “jerky” bifid (spike-

and-dome pulse)

Harsh systolic ejection murmur across entireprecordium→ apex & heart base

MR: separate murmur: severity of MR related todegree of outf low obstruction



EKG:



Echocardiography:

2D-echo:

Asymmetric septal hypertrophy

Diffuse concentric or localized to apex/anterior wall

Systolic anterior motion of MV (SAM)



Doppler Echocardiocraphy:

Typical appearance: late-peaking signal “dagger-shaped”

Bernoulli for peak systolic gradient(+ maneuvers)

Obstructive or non-obstructive

Distinguish MR and intra-cavitary obstruction

(looking for the aortic closure signal)



Cardiac cath:

Not necessary




↑ LV systolic pressure

↓ Ao systolic pressure

↑ gradient between LV & Ao

Post PVC



Imitator of HCM

Amyloidosis:

Thickened walls & low voltage on EKG.



Natural history of HCM

Mortality: 3 %/year (6-8 % with NSVTach)

Poor prognosis:

- Younger age

- Male sex

- + family hx. of sudden death

- Hx. of syncope

- Genetic markers (mutations of arginine gene)

- Exercise-induced hypotension (worst)



Genetic defect and prognosis



Management

All first degree relatives: screening…

echocardiography/genetic counseling

Avoid competitive athletics

Prophylactic antibiotics before medical & dentalprocedures

Holter x 48 hours



β- Blockers: Propranolol 200-400 mg/d(large doses)/ Selective β- B lose selectivity at highdoses:

Slow HR → longer diastolic filling time → ↓ myocardial O2 consumption →

↓ myocardial ischemia & LVOT obstruction

CaCh- Blockers: Verapamil 240-320 mg/d

(with caution for hemodynamic deterioration)

Combination of both



Disopyramide: class I antiarrhythmic + strong –iveinotropic effect

Non-responders to Medical therapy

???



???

1- Surgery (Myotomy/Myectomy) +/- MVR

2- ICD

3- DDD pacemaker

4- NSRT (alcohol septal ablation)



1- Surgery:

Septal myotomy/myectomy:

Patients < 40 years: mortality < 1 %

Patients > 65 years: mortality 10-15 %

Survival better than medically treated patients Should be considered in: resting gradient > 50 mmHg, or

refractory to medical Rx.

Young patients, particularly those with severe disease

Additional structural abnormalities affecting the mitral valve or coronary arteries.

Complication (rare): Aortic incompetence



Myotomy/Myectomy



2- ICD:

Previous sudden death

High risk of sudden death

EPS use ?



3- DDD pacemakerSubstantial ↓ gradient(~ 50 %)



Effect of DDD pacemaker in HCM



Potential Mechanisms of benefit of Pacing in HCM:



RV apical pacing & maintenance of AV synchrony → abnormal pattern of septal contraction → ↓ early systolic bulging of hypertrophic subaortic septum inLVOT &

↓ Venturi forces that produce SAM.

↑ LVOT width during systole

↓ systolic hypercontractility: ↑ end-systolic volume→ ↓ intraventricular pressure gradients & myocardial work



Candidates for DDD

4 Alcohol septal ablation (NSRT)



4- Alcohol septal ablation (NSRT)

Controlled myocardial infarction

of the basal ventricular septum

to ↓ gradient.

First septal artery occluded with

a balloon catheter and ETOH

injected distally



NSRT (Non Surgical Septal Reduction Therapy)The most appropriate candidates for NSRT should meet all of thefollowing criteria :

- HCM with severe symptoms of heart failure (NYHA class III to IV)despite adequate tolerated drug therapy

- An LVOT gradient 50 mmHg at rest or after exercise or >30 mmHg atrest or 60 mmHg under stress

- Basal septal thickness 18 mm

- NYHA class II heart failure with a resting LVOTgradient >50 mmHg or>30 mmHg at rest and 100 mmHg with stress .

- Elderly or comorbidities that may increase the risk of surgicalcorrection.



DEFINITION



WHO• RCM as a myocardial disease characterized by

restrictive filling and reduced diastolic volume of either or both ventricles with normal or near-normal

systolic function and wall thickness.

• The hallmark of the restrictive cardiomyopathies is

abnormal diastolic function; the ventricular walls areexcessively rigid and impede ventricular filling.



Types – based on etiology



yp gy

Primary or Idiopathic

Familial

Secondary Amyloidosis (commonest)

Sarcoidosis

Endomyocardial fibrosis

Symptoms• Gradually worsening shortness of breath, progressive



exercise intolerance, and fatigue. This exertional dyspnea

reflects the left heart failure.

• Fatigue and weakness are results of the decreased stroke volume.

• Patients may have distention of the abdomen andbilateral swollen feet (right heart failure).

• Angina like chest pains are observed only in patients withamyloidosis.

Symptoms• Patients may complain of palpitations (atrial fibrillation),



which are common in idiopathic RCM.

• As many as one third of patients with idiopathic RCMmay present with thromboembolic complications.

• Patients may have a history of syncopal attacks.

• Conduction disturbances particularly are common ininfiltrative RCM.

• Depending on the etiology, patients may have a priorhistory of radiation therapy, heart transplantation,chemotherapy, or a systemic disease.



SignsGPE

Px may be more comfortable sitting

Px may have minimal to moderate ascites and pitting

pedal edema

CVS Exam The pulse is low volume, consistent with decreased

stroke volume.



Signs• High JVP with diastolic collapse (Friedreich's sign).

• JVP with rapid X and Y descents, but the mostprominent wave is the Y descent (atrium emptyinginto the “stiff” ventricle)

• Elevation of JVP with inspiration (Kussmaul's sign).

• S4 in early disease (forceful atrial contraction against astiff ventricle).

• S3 in advanced disease.• Murmurs due to mitral and tricuspid valveregurgitation may be present.



SignsResp. Exam.

Reduced breath sounds because of pleural effusion

Crepitations due to left heart failure

Abd. Exam.

In advanced cases, liver may be palpable and pulsatile.



The goal of treatment in RCM is to reduce symptomsby lowering elevated filling pressures withoutsignificantly reducing the cardiac output.

i i



Investigations• CXR

– Pulmonary venous congestion .The cardiac silhouettecan be normal (familial) or show cardiomegaly and/oratrial enlargement.

• ECG – usually has low-voltage and ST segment and T wave

abnormalities.

• Echocardiogram – symmetrical myocardial thickening and often a normal

systolic ejection fraction, but impaired ventricularfilling.



Cardiac catheterization and haemodynamic studieshelp distinction from constrictive pericarditis.

Endomyocardial biopsy in contrast with othercardiomyopathies is often useful in this condition andmay permit a specific diagnosis such as amyloidosis to

be made.

DIFFERENTIAL DIAGNOSIS



DIFFERENTIAL DIAGNOSISConstrictive Pericarditis

Pericardial calcification on x-ray, which occurs inconstrictive pericarditis, is absent.

Right ventricular transvenous endomyocardial biopsy (by revealing myocardial infiltration or fibrosis inrestrictive cardiomyopathy)

CT scan or MRI (by demonstrating a thickened

pericardium in constrictive pericarditis).

pericarditis and restrictivecardiomyopathy



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Clinical Features Constrictive Pericarditis RestrictiveCardiomyopathy

History Prior history of pericarditisor condition that causes

pericardial disease

History of systemic disease(eg, amyloidosis,

hemochromatosis)

General examination Peripheral stigmata of systemic disease

Systemic examination -Heart sounds

Pericardial knock, high-frequency sound

Presence of loud diastolicfilling sound S3, Low-

frequency soundMurmurs No murmurs Murmurs of mitral and

tricuspid insufficiency

Prior chest x-ray Pericardial calcification Normal results of priorchest x-ray

TREATMENT & PROGNOSIS



TREATMENT & PROGNOSIS There is no specific treatment.

Cardiac failure and embolic manifestations should be

treated.

Cardiac transplantation should be considered in somesevere cases, especially the idiopathic variety.

In primary amyloidosis, combination therapy withmelphalan plus prednisolone with or without

colchicine may improve survival



However, patients with cardiac amyloidosis have a worse prognosis than those with other forms of thedisease, and the disease often recurs after

transplantation.

Liver transplantation may be effective in familial

amyloidosis (due to production of mutantprealbumin) and may lead to reversal of the cardiacabnormalities.

Cardiomyopathy.pptx2!5!13 3 Year Mbbs

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