4. Epidemiology
Incidence: 1:1,00,000
1% of all pediatric cardiac disease
During infancy incidence is 10 times
higher than older children
40% die within 2 years of life
Idiopathic is 70-80%
5. Cardiomyopathy:
Pathophysiologic Classification
Dilated Cardiomyopathy
Insult to the myocardium
tissue necrosis/interstitial fibrosis
impaired systolic
contractility/diastolic compliance
ventricular dilation to maintain
function
Left +/- right sides
Hypertrophic Cardiomyopathy
Myocyte hypertrophy & disarray
Increased mass & thickness
Increased mass/volume ratio
Poor diastolic chamber compliance
Left ventricle
High systolic pressure gradient
Restrictive Cardiomyopathy
Rare, very small L ventricular cavity
Impaired diastolic function initially
Unclassified cardiomyopathy
10. DCM: History
Insidious onset, may be acute in up to 25% of patients, exacerbated
by a complicating LRTI
Cough, poor feeding, irritability, and shortness of breath are usually the
initial presenting symptoms.
Pallor, sweating, easy fatigability, failure to gain weight, and decreased
urine output may be present.
Wheezing may be an important clinical sign, suggesting
congestive heart failure (CHF) manifestation in infants.
Chest pain, palpitations, orthopnea, hemoptysis, frothy sputum, sudden
death, abdominal pain, syncope, and neurologic deficit are other
modes of presentation (20%).
Cardiomegaly detected incidentally on a chest radiograph or an
arrhythmia detected incidentally on an ECG may be the basis for initial
cardiac referral.
Approximately 50% of patients with dilated cardiomyopathy (DCM)
have a history of preceding viral illness. A detailed family history for
familial cardiomyopathy is revealing in up to 25% of cases.
11. DCM: physical findings
In established disease, features of CCF are dominant.
Major cardiac findings include cardiomegaly, quiet
precordium, tachycardia, gallop rhythm (S3 and/or
S4), accentuated P-2, and murmurs of mitral and
tricuspid regurgitation. Murmurs may be
inconspicuous initially when presenting in acute heart
failure.
Infants often present with predominantly respiratory
signs and, in the absence of a precordial heave or
prominent murmur, the underlying cardiac disease
may remain undiagnosed until cardiomegaly is
detected on chest radiograph.
13. ECG
Presence of Q waves and inversion of T
waves in leads I, II, aVL, and V4
through V6 (anterolateral infarction
pattern): ALCAPA
Significant arrhythmia: Arrythmia
causing DCM
Low Voltage complexes: Pericardial
effusion
14. ECHO
Dilated left ventricle (>95th percentile) with
global hypokinesia (fractional shortening
<25%, ejection fraction <50%), and no
demonstrable structural heart disease: DCM
Left ventricular posterior wall hypokinesia
with hyper-echoic papillary muscles,
retrograde continuous flow into proximal
pulmonary artery: ALCAPA
Significant pericardial effusion with
satisfactory left ventricular ejection fraction:
Pericardial effusion
15. Cardiomyopathy: Management
Supportive Therapy
Non specific therapy for heart
failure, to improve survival &
alleviate symptoms
ACE inhibitors (captopril, enalpril)
Reduce afterload
Improve cardiac ejection
Reduce catecholamine drive
prolonging cardiac survival
Careful titration necessary
B blockers (metoprolol, carvedilol)
Digoxin
Diuretics
Specific Therapy
Depends on the underlying disease
condition
Most have no effective Rx
Carnitine supplements
Surgery
Correction of aberrant vessels
Implanable defibrillators
Partial left venticulectomy
Cardiac transplant
20. Alteration of afterload
Precaution: Do not use in hypovolumic
condition and in pt with fixed left ventricular
outflow obstruction
Effective in Regurgitant lesions(ECD,
Cardiomyopathy) and left to right shunts
(VSD)
Acute: Nitroprusside, Dobutamine, amrinone
Chronic: ACE inhibitors
Enalapril: 0.1 mg/kg /day OD or BD ( 5 kg: ¼ tab
OD)
21. ACE inhibitors
Captopril:
Neonate: 0.1 –0.4 mg/kg/dose 1-4 times a
day
Infant: 0.1 – 1 mg /kg/dose 1-4 times a
day
Child: 12.5 mg/dose 1-2 times a day
Enalapril: 0.1 mg/kg 1-2 times a day
never >0.5 mg/kg/day
22. K concerns
If using Furesamide >2mg/kg/day
add oral potassium
Add spironolactone
If using ACE inhibitors do not use
spironolactone
Electrolytes should be monitored monthly
Hyponatremia should be managed with
decreasing diuretic and restricting fluid, not
by supplementing sodium
25. Role of beta blocker
Adrenergic stimulation happens in CCF
Increases HR and contractility
Alpha stimulation leads to peripheral and
coronary constriction, increase O2 demand
and after load
Beta1 receptor stimulation causes calcium
accumulation in cells and cell death
Carvedilol is beta and alpha blocker
26. K concerns
If using Furesamide >2mg/kg/day
add oral potassium
Add spironolactone
If using ACE inhibitors do not use
spironolactone
Electrolytes should be monitored monthly
Hyponatremia should be managed with
decreasing diuretic and restricting fluid, not
by supplementing sodium
29. Restrictive Cardiomyopathy
Restriction of diastolic filling
Causes: amyloidosis, hemosiderosis,
hypereosinophilia, and endocardial
fibroelastosis
Treatment: unhelpful, Only diuretic
30. MI in children
ALCAPA
Post TGA operation: coronary ostia
stenosis, kinking of coronary artery
Thrombotic occlusion in KD
Takayashu arteritis
SCD
31. Aspirin in KD
Acute intervention for Kawasaki disease:
80-100 mg/kg/d PO divided q6h until afebrile
for 2-3 d
Subsequent antiplatelet dose:
3-5 mg/kg/d PO
Duration of treatment is 6-8 wk from onset of
illness or until erythrocyte sedimentation rate
and platelet count return to reference range
may require indefinite continuation if
coronary artery abnormalities are observed
32. MI in ALCAPA
Infant develops irritability with dyspnea, tachycardia,
diaphoresis, and vomiting while feeding. Irritability is
secondary to anginal pain caused by a coronary artery steal
phenomenon to the anomalous origin of the left coronary
artery. The flow in this vessel, which has its distribution over
the left ventricular myocardium, is retrograde to the main
pulmonary artery.
The diagnosis of ALCAPA is suspected in irritable anxious
infants presenting with pain while feeding. ECG
demonstrates classic findings of deep Q waves, peaked T
waves, and/or ST segment changes consistent with
ischemia, injury, or infarction.
33. MI in KD
Coronary artery involvement occurs in 15-25% of
children with Kawasaki disease within 1-3 weeks of
onset. In patients with untreated Kawasaki disease,
sudden death has resulted from acute myocardial
infarction caused by ruptured coronary artery
aneurysms or thromboses.
Detrimental changes in arterial wall hemodynamics
are present and persist after acute Kawasaki disease
which may predispose to long-term cardiovascular
events.
36. Neonatal Cardiomyopathy:
Asphyxia induced
Hypoxia leads to myocardial ischemia/dilation
Term infant with delivery complicated by hypoxic stress
Apgars usually <3 @ 1
Metabolic acidosis/ multi system ischemia
Severe cases: Hypotension/shock
Murmur of mitral/tricuspid regurg may be present
EKG: Diffuse ST -T changes, R atrial hypertrophy
Prognosis: Good without cardiogenic shock
37. Neonatal Cardiomyopathy:
From Maternal Diabetes
Asymmetric hypertrophic cardiomyopathy
Mechanism not clearly understood ? Hyperinsulinemia
Prevalence unrelated to diabetic control of mother
Puffy, Plethoric infant, with signs and symptoms of CCF
SEM common and related to degree of outflow obstruction
RX:Usually symptomatic
Prognosis: Usually good, resolves in months
Digitalis and other inotropics agents are contraindicated
except in very severe depression of myocardial contractility
38. Neonatal Cardiomyopathy:
Carnitine deficiency
Autosomal recessive inheritance
Plasma memb carnitine transport defect: Impairs fatty acid oxidation
Metabolic acidosis, intractable hypoglycemia, severe non-immune hydrops, +/-muscle
weakness
EKG: Giant T waves(pathognomonic)
Subnormal carnitine level 1-2 %, heterozygous parents have 50 % levels
Symptomatic Rx for the cardiac failure gives minimal benefits
Definitive Rx: Oral carnitine supplements
Prognosis: Usually good with early diagnosis and Rx
Risk of growth and mental retardation
39. Neonatal Cardiomyopathy:
Myocarditis
Any infectious agent, commonly Coxsackie B, ECHO viruses, herpes, HIV, Rubella
Bacterial/fungal infections
Vertical/horizontal spread
Pathology: multicellular infiltrates
Usually first 10 days of life
Features of acute infective process
Involvement of other organs like CNS esp Coxsackie B
Gamma globulins beneficial
Rx underlying infection: Interferon, Ribavirin
40. Neonatal Cardiomyopathy:
Pompe’s Disease
Generalized form of glycogen storage dse (type II)
Lysosomal alpha- glucosidase deficiency
Autosomal recessive
Infiltrative cardiomyopathy
Skeletal muscular hypotonia: Protruding tongue, feeble cry, poor feeding
Hyporeflexia
Diagnosis: Measurement of enzyme activity or DNA analysis
EKG: (characteristic)
Short PR interval
prominent P waves
massive QRS voltage
Uniformly fatal
41. Neonatal Cardiomyopathy:
Endocardial Fibroelastosis
No established cause
Also called elastic tissue hyperplasia
Pathology: White opaque fibroblastic thickening of the endocardium
1:6000 (1960); 1:70,000 (1980)
Infants < 6 months usually
Severe CCF/ rhythm disturbances
Failure to thrive
CXR : Massive cardiomegaly
EKG: Low voltage as in severe myocarditis
ECHO: Bright -appearing endocardial surface
42. Neonatal Cardiomyopathy:
Anomalous origin of the left coronary artery
From the pulmonary artery
Should be ruled out in all cases of cardiomyopathy
EKG: anterolateral infarct
Surgical correction usually successful
