Cardiomyopathy in neonates and children

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Cardiomyopathy in neonates and children

• Dr Rajesh Kumar
• MD (PGI), DM (Neonatology) PGI, Chandigarh, India
• Rani Children Hospital, Ranchi

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• Some cardiomyopathies are treatable
• Cardiomyopathy may presents as recurrent wheeze

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Classification of Cardiomyopathy

• Dilated
• Chronic
• Acute Viral myocarditis (Inflammatory
  Cardiomyopathy)
• Hypertrophic
• Restrictive

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Epidemiology

• Incidence 11,00,000
• 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

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CardiomyopathyPathophysiologic 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

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Causes of dilated Cardiomyopathy
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Causes of dilated Cardiomyopathy
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(No Transcript)
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Pathogenesis of idiopathic DCM

• preceding viral myocarditis
• autoimmunity
• Underlying genetic predisposition

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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.

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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.

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Diagnostic Evaluation

• Step 1 Initial Evaluation
• EKG
• CXR
• ECHO
• Step 2 Screening Evaluation
• CBC
• EnzymesSGOT, SGPT, CPK,
• ABG
• Fractionated serum carnitine
• Urine organic amino acids
• Urine muco/oligosacharides
• Skeletal survey

• Step 3 Specific Testing
• Cardiac catheterization
• Myocardial biopsy
• Holter monitoring
• Carnitine levels (skeletal, cardiac tissue,
  urine)
• Serum ketone bodies, ammonia, pyruvate, lactate
• Fibroblast studies
• Chromosomes

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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

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ECHO

• Dilated left ventricle (gt95th percentile) with
  global hypokinesia (fractional shortening lt25,
  ejection fraction lt50), 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

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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

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Digoxin

• Inotropic agent
• Loading dose
• Premature neonate20-30 mg/kg
• Term neonate 30-40 mg/kg
• Schedule for loading ½, ¼, ¼ 8hours apart
• Maintanance dose
• Premature neonate 5-10 mg/kg/day BD
• Term neonate 10 mg/kg/day BD

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Digoxin

• Route IV, IM, oral
• Injection 1ml ampoule, 250 mg /ml
• 1unit 6.25 mg 10 mg /kg 1.5units/kg
• Oral (Digoxin Paed elixir) 1ml 0.05 mg
• Maintenance dose 0.01 mg/kg/day
• Wt in kg /10 ml twice daily
• 3 kg 0.3 ml twice daily

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Alteration of preload

• Fluid retention due to low cardiac output and
  renal perfusion
• Ventricular contractility is compromised due to
  massive volume overload
• Diuretics
• Acute diuresis Furosemide 1-4 mg/kg/dose
• Chronic diuresis Furosemide potassium sparing
  diuretics

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Maximum diuretic therapy

• Frusamide upto 2mg/kg/dose TDS
• Frusamide Thiazide diuretic
• Frusamide Metolazone
• Metolazone 0.2 mg/kg/dose OD
• Hydrochlortiazid 2-4 mg/kg/day
• Chlorthiazide 20-40 mg/kg/day

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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)

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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 gt0.5
  mg/kg/day

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K concerns

• If using Furesamide gt2mg/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

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Carvedilol in cardiomyopathy
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Tab CARDIVAS 3.125 mg
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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

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K concerns

• If using Furesamide gt2mg/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

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Treatment

• Carnitine 25-50 mg / kg/dose BD or TDS, Max
  200mg/kg/day
• Coenzyme Q10 variable result

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Hypertrophic Cardiomyopathy

• Clinical Sudden death, Syncope, Presyncope,
  dizziness, palpitation
• Murmur, S3
• ECG Arrythmia
• ECHO septal thickness is gt 1.4 times the
  posterior wall thickness
• Treatment propanalol, Verapamil, amiodarone

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Restrictive Cardiomyopathy

• Restriction of diastolic filling
• Causes amyloidosis, hemosiderosis,
  hypereosinophilia, and endocardial fibroelastosis
  
• Treatment unhelpful, Only diuretic

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MI in children

• ALCAPA
• Post TGA operation coronary ostia stenosis,
  kinking of coronary artery
• Thrombotic occlusion in KD
• Takayashu arteritis
• SCD

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Aspirin in KD

• Acute intervention for Kawasaki disease80-100
  mg/kg/d PO divided q6h until afebrile for 2-3 d
• Subsequent antiplatelet dose3-5 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

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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.

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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.

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Neonatal Cardiomyopathy
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Neonatal Cardiomyopathy Etiologic
classification

• HYPERTROPHIC
• Familial
• Idiopathic Hypertrophic
• Maternal disease
• Diabetes
• Myocarditis
• Infectious
• endotoxins, exotoxicins
• Drugs /Iatrogenic
• Dexamathasone (BPD)( case report)
• ECMO (case report)
• Adriamycin
• Chloramphenicol
• Malformation syndromes
• Beckwith wiedemann

• DILATED
• Perinatal insult/ maladjustment
• Asphyxia
• Persistent fetal circulation
• Congenital anomalies
• Anomalous origin of Left coronary
• Inborn errors of metabolism
• Glycogen storage dses (Pompes dse)
• Mucopolysaccharidosis
• Disorders of fatty acid metabolism (Carnitine
  deficiency)
• Amino organic acidiurias
• Maternal connective Tissue dse
• SLE

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Neonatal Cardiomyopathy Asphyxia induced

• Hypoxia leads to myocardial ischemia/dilation
• Term infant with delivery complicated by hypoxic
  stress
• Apgars usually lt3 _at_ 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

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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
• RXUsually symptomatic
• Prognosis Usually good, resolves in months
• Digitalis and other inotropics agents are
  contraindicated
• except in very severe depression of myocardial
  contractility

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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

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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

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Neonatal Cardiomyopathy Pompes 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

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Neonatal Cardiomyopathy Endocardial
Fibroelastosis

• No established cause
• Also called elastic tissue hyperplasia
• Pathology White opaque fibroblastic thickening
  of the endocardium
• 16000 (1960) 170,000 (1980)
• Infants lt 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

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Neonatal CardiomyopathyAnomalous 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