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CARDIOMYOPATHY
ANDMYOCARDITIS
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

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

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
DYSFUNCTIO
N
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-adrenoreceptor
blockers(+/-)
-Anticoagulants
antiarrythmicsCardiac
transplantation
Diuretics
-Anticoagulants(+/-)-
corticosteroids(+/-)-
permanent pacemaker for
advanced heart blockCardiac
transplantation

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

[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

Chest X-ray

Echocardiography

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 I
Mainly 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 AED

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

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