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CARDIOMYOPATHY AND MYOCARDITIS

Guided by : Dr Sandeep agarwal

Presented by : Dr Abhay Pota

www.dnbpediatrics.com

Table of Contents

[A] CARDIOMYOPATHY

1.HOCM

2.DCM

3.RCM

4.LVNC

5.ARVC

6.EFE

[B] MYOCARDITIS


[A] CARDIOMYOPATHY DCM

i) NMD – muscular dystrophies (duchene, becker, emery dreifuss, limb girdle etc)

- myotonic dystrophy

- myofibrillar myopathy

ii) IEM – fatty acid oxidation disorders

- carnitine abnormalities (CPT I, CPT II)

- mitochondrial disorders (kearns-sayre)

- organic acidemias (propionic acidemia)

iii) genetic mutation in cardiomyocyte structure

iv) genetic syndromes – alstrom syndrome, barth syndrome

v) ischemia – more common in adults

vi) chronic tachyarrythmias


(2) HCM

i) IEM – mitochondrial disorders (freidrich ataxia), storage disorders

( esp. pompe, MPS, fabry, sphingolipidosis, hemochromatosis)

ii) genetic mutation in cardiomyocyte structure

iii) genetic syndromes- noonan, costello, cardiofaciocutaneous, beckwith- wiedemann

iv) infant of a diabetic mother


(3) RCM

i) NMD- myofibrillar myopathies

ii) metabolic – storage disorders

iii) genetic mutations in cardiomyocyte structure


iv) Arrythrogenic right ventricular cardiomyopathy

a) genetic mutation in cardiomyocyte structure

v) LV non compaction

b) X-linked, AR, AD, mitochondrial, sporadic


[B] secondary or acquired myocardial disease

(1) MYOCARDITIS

i) viral

parvo bilateral, adeno, coxsackie A & B, echo, rubella, varicella, influenza, mumps

EBV, cytomegalovirus, mumps, measles, polio, Hepatitis C, HIV,

ii) rickettsial – psittacosis, RMSF, typhus, coxiella burnetti

iii) bacterial – diptheria, mycoplasma, meningococci, leptospira, lyme, typhoid, TB,

streptococci

iv) parasites – chagas, toxoplasma, cysticercosis, schistosomia

v) fungal – actino, coccidiomyco, histoplasmo


(2) systemic inflammatory disease

SLE, scleroderma, churg-strauss RA, RF,

sarcoidosis, dermatomyositis, loffles

(3) Nutritional deficiency

Beri beri, kwashiorkor, keshan

(4) drugs

doxorubicin, chloroquine, cyclophosphemide, sulphonamides,

alcohol, irradiation, herbal remedy


(5) CAD – kawasaki, ALCAPA, familial hypercholesterolemia

(6) Hemato-oncology – anemia, leukemia, SCD

(7) endocrine – neuroendocrine – hyperthyroidism,

carcinoid chromocytoma


HOCM

It is a genetic disorder

Most common are mutations in genes encoding Cardiac B myosin

heavy chain (MYH7)Myosin binding protein C (MYBPC3)

AD pattern of inheritance

Other are PRKAG2 & LAMP2 α- galactosidase mutations


PATHOGENESIS

Characterized by increased LV thickness in the absence of structural heart disease

or hypertension

Inter-ventricular septum is often disproportionately involved (IHSS) or (ASH).

In some patients intra-cavitary gradiant develops during systole.

This subaortic obstruction is caused by systolic anterior motion of mitral valve

against hypertrophied septum

Diastolic ventricular filling is impaired by abnormal stiffness of LV, which leads to LAH

and pulmo venous congestion, producing congestive symptoms


a large volume of stroke volume (80%) is ejected during the early part of systole

when there is little or no obstruction, producing a sharp upstroke in the

arterial pulse- characteristic of HOCM

Because the obstruction of LVOT results from SAM of mitral valve against

hypertrophied septum, any influence that reduced LV systolic volume

(+ve inotropic agents, lowering of SVR. Low blood volume) will increase

obstruction and increase the murmur, while any influence that increases the

LV systolic volume (-ve inotropic agents, Leg raising, blood transfusion)

will decrease obstruction and decrease the murmur


Clinical features

H/O : many patients asymptomatic

-50% cases present with heart murmur or detected

during family screening

-palpitation , chest pain, easy fatiguability,

-dyspnea, dizziness, syncope

-sudden death


P/E

– over active pre-cordial impulse with a heave

abnormal PP

- Systolic ejection murmur in the aortic region

not associated with ejection click

- A sharp upstroke on arterial pulse


ECG

– LVH, ST-T changes, abnormally deep Q waves

WPW syndrome in Danon and Pompe diasease

X-ray

- mild LVH with globular heart

- pulmo vascularity normal


ECHO

Z Score of 2 or more relative to BSA is diagnostic

LV wall thickness Doppler helps in defining,

localising, quantifying the degree of myocardial

hypertrophy and also demonstrates degree of

LVOT and mitral insufficiency

Cardiac catheterization

Metabolic testing

Genetic testing www.dnbpediatrics.com

MANAGEMENT 1) sternous activity prohibited and rest

2) B blockers like propranolol or atenolol or CCBs

like verapamil, useful in diminishing LVOT,

modifying ventricular hypertrophy and

improve ventricular filling.

- Though, risk of sudden death not decreased.

3) antiarrythmic treatment in patient with atrial or ventricular

arrythmias


4) patients with documented ventricular

arrythmias, strong family history or patients

with syncope – ICD

5) dual chamber pacing, alcohol ablation,

surgical septal myomectomy, MVR – limited success

6) first degree relatives should be screened every

3-5 years for patients under 12 years of age and

then yearly throughout the teenage and young adulthood


PROGNOSIS

Children <1 year, with IEM or with some syndromes have poorer prognosis

Risk of sudden death is increased in patients with a History of cardiac arrest,

History of Vtach, Exercise hypotension, syncope>3 cm wall thickness,

F/H/O sudden death


DCM

Most common form of cardiomyopathy

Most common cause of DCM is idiopathic

Among familial causes AD is most common


PATHOLOGY AND PATHOPHYSIOLOGY

Weakening of systolic contraction

Dilatation of all 4 chambers

Dilatation of atria is in proportion to ventricles

(unlike RCM)

Intracavitary thrombus formation is common

in apical part of ventricles Pulmo& systemic emboli

On biopsy, myocyte hypertrophy and fibrosis


CLINICAL FEATURES

history of – fatigue, weakness, dyspnea, orthopnea

P/E – s/o CHF (increased heart rate, crackles,

weak PP, distended neck veins, heapatomegaly)

Gallop rhythm, murmurs of MR or TR


ECG

sinus tachycardia,

LVH & ST-T changes most common LAH,

RAH

Atrial or ventricular arrythmias

AV conduction disturbances


x ray & echo

Cardiomegaly with or with out pulmonary edema

Echo – most important tool in diagnosis

pulmonary hypertension,

MR or other structural defects.


Additional testing

CPK

KFT

LFT

Troponin

Lactate

plasma amino acids

urine organic acidsacylcarnitiine profile www.dnbpediatrics.com

MANAGEMENT

[1] medical treatment

Aimed at treating underlying heart failure

Diuretics, digoxin, ACEI, ARB

Bed rest and restriction of activity

Inotropic support and mechanical ventilation


[2] B adrenergic blockade with carvedilol or metoprolol

Pediatric data not available

[3] ECMO, ventricular assist devices, cardiac transplantation

[4] specific antiarrythmic therapy and anticoagulation


PROGNOSIS

1 and 5 year rates of death or need for transplantation in DCM

patients is 31% and 46%

Independent risk factors for death or need for transplant in DCM

patients are Older age, CCF Lower left ventricular functional

shortening zone


RCM

<5 % of cases Incidence increases with age,

M<F Most common etiology is idiopathic


PATHOGENESIS

Normal ventricular chamber diameter Normal ventricular wall

thickness Preserved systolic function Impaired ventricular filling

Normal size of ventricles with dilated atrias


Clinical features

H/O : exercise intolerance, weakness, dyspnea, chest pain

P/E : jugular venous distention, gallop rhythm,

systolic murmur of AV valve regurgitation

ECG : bilateral AH, AV block

X-ray : cardiomegaly, pulmonary venous congestion


ECHO : normal LV systolic function

diastolic dysfunction

bilateral AH with normal LV dimension

Cardiac catheterisation :

PApressure & RV & LV

EDP Increased

Endomyocardial biopsy


MANAGEMENT

1)Treatment of CCF

2) CCB to increase diastolic compliance

3) anti-coagulants & anti-platelets to prevent thrombus

4) pacemaker for CHB5) cardiac transplantation


SUMMARY OF CLINICAL CHARACTERISTICS OF CARDIOMYOPATHY

CLINICAL FEATURES

HYPERTROPHIC DILATED RESTRICTIVE

CAUSE Inherited (AD in about 50%)Sporadic(new mutation)

Pluricausal (e.g.toxic metabolic, infectious, alcohol, doxorubicin

Myocardial fibrosis, hypertrophy, or unfiltration (amyloid hemochromatosis)

HEMO-DYNAMIC DYSFUNCTION

Diastolic dysfunction (with normal systolic function) (abnormally stiff LV with impaired ventricular filling

Systolic contractile dysfunction (decreased cardiac output, decreased stroke volume, increased LVEDP)

Diastolic dysfunction (rigid ventricular walls impede ventricular filling


CLINICAL FEATURES HYPERTROPHIC DILATED RESTRICTIVE

Echo (morphology) Thickened LV (and occasionally RV) wall -Small or normal LV chamber dimensionsSupernormal LV contractility HOCM and/or ASH

Biventricular dilatation (increased LVDD and LVSD)-atrial enlargement in proportion to ventricular enlargement -Decreased LV contractility Apical thrombus(+/-)

Biatrial enlargement -Normal LV and RV volume Normal LV systolic function until advanced stage Atrial thrombus(+/-)

Doppler Reduced relaxation pattern Reduced relaxation pattern ‘reestrictive’ pattern

Treatment B-adrenoreceptor blockers -calcium antogonists(digitalis/catechols and nitrates contraindicated)- (diuretics may worsen the symptoms)

Vasodilator therapy -digitalis plus diuretics B-adrenoreceptorblockers(+/-)-Anticoagulants antiarrythmicsCardiactransplantation

Diuretics-Anticoagulants(+/-)-corticosteroids(+/-)-permanent pacemaker for advanced heart blockCardiactransplantation


4. LVNC Left ventricular non compaction

affects all ages

Trabeculated or spongy appearing LV,

commonly a/w LVH or LV dilatation, and, at times systolic or diastolic dysfunction

ECG – LVH, ST-T changes or arrythmias

MRI – trabeculated LV myocardium,

characteristically within apex of left ventricle

Metabolic screen – especially in young patients TA2 gene mutation


TREATMENT

anticoagulation, anti-arrythmic treatment if needed &

treatment of HF if present

Cardiac transplantation in patient refractory to medical treatment


5. ARVC

-Arrythmogenic right ventricular cardiomyopathy

-AD from most common

-AR forms a/w skin manifestations

-Characterized by dilated RV with fibrofatty

-infiltration of RV wall

-Global and regional right and left ventricular dysfunction and

-ventricular tachyarrythmias

-Syncope or sudden death can be the presentation

-Mx - antiarrythmics, defibrillation and Mx of HF, Cardiac transplantation www.dnbpediatrics.com

[6] EFE

endocardial fibroelastosis

Incidence markedly decreased due to abolition of mumps virus inf by immunisation

Characterized by opaque, white, fibroelastic thickening on the endocardial surface

of the ventricle, which leads to systolic or diastolic dysfunction

Mx of HF & cardiac transplantation


MYOCARDITIS


Definition and Etiology

Process characterized by inflammatory infiltrate of the

myocardium with necrosis and/or degeneration of adjacent

Myocytes, not typical of the ischemic damage associated with

coronary artery disease

Most cases from common viral infections and post-viral immune

-mediated responseCoxsackievirus A and B, echovirus, poliovirus,

PVB 19, HHV6Precursor of dilated cardiomyopathy


Etiology


Time Course of Viral Myocarditis


Incidence

Often not recognized

Estimated annual incidence of 1/100.000

4 – 5% in young accident victims

12% in adolescents and young adults with sudden cardiac death


Clinical features

H/O : URTI in older children or may have a sudden onset with

anorexia , vomiting, lethargy.

In new borns and infants signs of CHF like tachycardia, tachyapnea,

gallop rhythm, and arrythmia may be present.

A soft systolic heart murmur and supra ventricular or

ventricular ectopic beats may be present


Laboratory Tests

Biomarkers (troponins, creatine kinase MB) occasionally elevated in childhood

(sensitivity 71%, specificity 86%)

Normal nonspecific markers of inflammation (CRP, leucocytes) do not exclude

acute myocardial inflammatory process

Utility of virus serology in patients with suspected myocarditis unproven –

costly and unreliable

• Detection of viral genome in urine or feces


ECG

Abnormalities present in 93-100%, but low sensitivity

Sinus tachycardia

Nonspecific T-wave and ST-segment changes

ST-segment elevation

AV conduction delays

Atrial and ventricular arrhythmias


ECG


Chest X-ray


Echocardiography


MRI


Endomyocardial Biopsy


Endomyocardial Biopsy

Is a Gold standard for diagnosis

Invasive, potential complications: pneumothorax, hemothorax, arrhythmias,

perforation, death

Enables identification of lymphocytic invasion and detection of involved virus

Poor sensitivity and specificity due to patchy myocardial inflammation

Substantial interobserver variation


Histology/Dallas Criteria

Acute myocarditis: lymphocytic infiltrate with myocyte necrosis

Borderline myocarditis: inflammatory infiltrate without necrosis

Chronic myocardits/DCM with inflammation:

>14 inflammtory cells/mm2 in the myocardium


Differential Diagnosis

Any disease with impairment of LV function

DCMA

LCAPA

Chronic tachycardia

Arteriovenous malformation


Therapy IMainly supportive, no trials for specific heart failure therapy in biopsy-proven

myocarditis in adults and children

Monitoring of vital parameters

Bed rest initially, no physical activities for 3-6 months

Trat heart failure therapy with ACE inhibitors, AT1-antagonists ß-blockers,

diuretics and aldosterone antagonists according to current guidelines

Ventricular assist device <-> HTX

Therapy of tachyarrhythmias in adults - Life vest and AEDwww.dnbpediatrics.com

Therapy II

In proven viremia: immunoglobulins 2 g/kg for 48 hours (Robinson JL 2005)

No immunosuppresive therapy – risk of enhanced virus replication and

blockade of endogenous interferons

Steroids only in biopsy-proven virus negative chroniC myocarditis (Frustacci A 2009)

Type-1 interferon (IFN-α, IFN-ß) beneficial in animal and human pilot studies

(Kühl U 2003, Schmaltz AA 1998)


Acute Congestive Heart Failure/Suspected Acute Myocarditis


REFERENCES

TEXTBOOK OF PEDIATRIC CARDIOMYOPATHY – S. PARKS 6th edition

NELSON – 19th editionE-medicine.medscape.com


www.criticalpediatrics.org

http://www.dnbpediatrics.com/

http://www.criticalpediatrics.org/

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