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CARDIOMYOPATHY

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Peripartum Cardiomyopathy: Implications to the fetal well-being ... Clinical adage 1/3rd die; 1/3rd significant damage; 1/3rd recover (infective myocarditis) ... – PowerPoint PPT presentation

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Title: CARDIOMYOPATHY


1
CARDIOMYOPATHY THE NEWBORN
  • N. Felicia Ochei, M.D.
  • Pediatrics-PL 2
  • November 2002

2
Introduction
  • Topics
  • Peripartum Cardiomyopathy Implications to the
    fetal well-being
  • Review of Cardiomyopathy in the Neonatal period
  • Fetal Cardiomyopathy A Journal article Review

3
Peripartum Cardiomyopathy
  • Definition
  • Dilated cardiomyopathy of uncertain origin
    characterized by
  • Cardiac failure in the last month of pregnancy or
    within 5 months after delivery
  • Absence of demonstrable cause for the cardiac
    failure
  • Absence of demonstrable heart disease before the
    last month of pregnancy
  • Documented systolic dysfunction

4
Peripartum Cardiomyopathy
  • Incidence
  • U.S. 11300 to 15,000 live births
  • Japan 16000 live births
  • South Africa 11000
  • Nigeria High incidence ? related to
    tradition of ingestion dried lake salt
  • Age
  • Wide range probably more common gt 30 years

5
Peripartum Cardiomyopathy
  • Medical Rx
  • Inotropics
  • Digioxin
  • Dobutamine when indicated
  • Loop diuretics
  • Beta blockers
  • Anticoagulation
  • Heparin(unfractionated, LMWH)
  • Warfarin (post partum)
  • After load reduction
  • Hydralazine
  • Nitrates
  • Obstetric mgt
  • Spontaneous vaginal delivery at term is
    reasonable unless mother is decompensating
  • Painless and effortless labor/delivery
  • Inhaled analgesia preferred (epidural/spinal
    contraindicated for 24hrs after use of LMWH)
  • Forceps/vacuum assisted delivery is the rule
  • Vaginal delivery preferred as C/S carries a
    higher risk of PE and and endometritis (75)

6
Peripartum Cardiomyopathy
  • Fetal Implications
  • Fetal distress from maternal hypoxia
  • Placental hypo-perfusion
  • Poor cardiac output
  • Excessive use of diuretics
  • Hypotension from afterload reducers
  • Complications of instrumental delivery
  • Complications of intra partum anesthesia (choice
    quantity)
  • Risks of Preterm delivery
  • Severe maternal decompensation
  • Adverse effects of medications (e.g. Digoxin,
    Beta blockers, LMWH)
  • Safety for use in pregnancy not established

7
Peripartum Cardiomyopathy
  • The pediatricians Role
  • Liaison with OB
  • Careful maternal history
  • Anticipate problems from
  • Preterm delivery
  • Maternal Medications
  • Fetal distress
  • Instrumental delivery

8
Neonatal Cardiomyopathy
9
Neonatal Cardiomyopathy
  • Definitions
  • Neonate Birth to 28 days of life
  • Neonatal Cardiomyopathy Disease of the neonate
    in which the myocardium is affected without
    primary abnormalities of the valves, great
    vessels or septum
  • Epidemiology
  • Difficult to define Few studies, rare disease
    entities
  • Estimates 1 10,000 live births (Nelson)
  • Constitutes about 1 of childhood cardiac disease
  • 10 of all pediatric cardiac deaths

10
Neonatal Cardiomyopathy Pathophysiologic
Classification
  • WHO (1980)
  • Guidance for therapy and prognosis
  • 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

11
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

12
Neonatal Cardiomyopathy Clinical Features
  • History
  • Non specific
  • Pallor, irritability
  • Tachypnea
  • Diaphoresis
  • Fatigue esp with feeds
  • Poor wt gain
  • PE
  • Signs of CCF
  • Tachypnea, tachycardia, narrow pulse p
  • Decreased peripheral pulse, hepatomegaly,
    wheezing
  • /- cyanosis
  • Murmur of mitral insufficiency
  • /- left ventricular outflow obstruction(hypertrop
    hic)
  • Features of underlying etiology
  • EKG
  • Flat T wave
  • ST depression
  • Generalized low voltages
  • Characteristic findings for the underlying
    abnormality
  • CXR
  • Cardiomegaly
  • May be normal in fulminant cases
  • Pulmonary edema
  • Pericardial effusion may be present (Water-bottle
    configuration)
  • ECHO
  • Diagnostic
  • Ventricular dilatation/dyskinesia
  • Ventricular outflow obstruction

13
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

14
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

15
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

16
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

17
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

18
Neonatal Cardiomyopathy 1diopathic Familial
  • Multi gene disorder
  • Autosomal with variable penetrance
  • Ventricular dysrhthmias/ Sudden death
  • Normal Echo _at_ birth does not rule out disease in
    later life
  • Avoid diuretics inotropics
  • Ventricular septal myomectomy
  • Cardiac transplantation
  • Those presenting _at_ birth have worse prognosis

19
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

20
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

21
Neonatal Cardiomyopathy Diagnostic Evaluation
  • Step 1 Initial Evaluation
  • EKG
  • CXR
  • ECHO
  • Step 2 Screening Evaluation
  • CBC
  • CMP
  • EnzymesLDH, SGOT, SGPT, CPK, aldolase
  • ABG
  • Fractionated serum carnitine
  • Urine organic amino acids
  • Urine muco/oligosacharides
  • Skeletal survey
  • Viral studies Stool, NPW, urine, blood
  • 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

22
Neonatal 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

23
Neonatal Cardiomyopathy Prognosis
  • Not well described in infants
  • Generally poor for infants
  • Depends on underlying condition
  • Some carry 100 mortality rate e.g. Pompe,s
    disease
  • Annual mortality 6 -8 in children
  • One year survival rate 63
  • 5 year survival rate
  • Clinical adage 1/3rd die 1/3rd significant
    damage 1/3rd recover (infective myocarditis)

24
FETAL CARDIOMYOPATHY
25
Fetal Cardiomyopathy A Journal Article Review
  • Schmidt KG, Einat B, Silverman NH, Scagneli SA.
  • Echocardiographic Evaluation of Dilated
    Cardiomyopathy in the Human Fetus
  • The American Journal of Cardiology 1989
    63599-605

26
Fetal Cardiomyopathy A Journal Article Review
  • Study Objectives
  • To explore the possibility of detecting dilated
    cardiomyopathy in the prenatal period
  • To follow the the development of the disease
    during gestation
  • To determine the effects of prenatal presentation
    on the postnatal course of the disease

27
Fetal Cardiomyopathy A Journal Article Review
  • Study Methodology
  • 625 women had fetal echocardiography at the Univ.
    of California in San Francisco from 1980 to 1987
  • Criteria for inclusion in the study
  • Family history of congenital heart defects
  • Abnormal findings in obstetrics sonogram
  • No history of antecedent maternal illness
  • The echo was performed from 20 to 26 weeks
    gestation for family Hx and _at_ time of
    presentation for the others

28
Fetal Cardiomyopathy A Journal Article Review
  • Study Findings
  • 6 of the 625 had dilated cardiomyopathy but had
    structurally normal hearts
  • 2 fetuses referred for family Hx had normal
    findings initially but later developed
    cardiomyopathy on serial ECHOs
  • Abnormal findings included
  • Reduced systolic myocardial performance(5)
  • AV valve regurgitation (3)
  • Abnormal chamber dimensions (3)
  • 4 deaths (1 fetus, 3 neonates) 1 survivor
    required cardiac transplant in infancy

29
Fetal Cardiomyopathy A Journal Article Review
  • Study conclusions
  • Dilated cardiomyopathy may develop during fetal
    life
  • Diagnosis can be achieved by serial
    echocardiogram
  • Normal findings in mid-trimester do not always
    rule out the subsequent development of
    cardiomyopathy
  • Reduced systolic performance most sensitive
    finding and preceded the presence of progressive
    dilation
  • Fetal onset cardiomyopathy carried poor prognosis
  • (Conflicts with other studies that suggested
    better outcomes for early childhood onset)
  • There were no predictive factors for outcome of
    the disease

30
Fetal Cardiomyopathy A Journal Article Review
  • Study Limitations
  • Technical limitations Unable to calculate
    ventricular volumes ejection fraction earlier in
    the study
  • Difficulty comparing chamber enlargements and
    performance with normal values
  • As was with all previous studies
  • No defined normal values

31
Fetal Cardiomyopathy A Journal Article Review
  • Discussion
  • The value of fetal echocardiogram in
    cardiomyopathy
  • Research and further development
  • Fetal echo usually done not solely for
    cardiomyopathy but for cardiac anomalies in
    general
  • Intervention ?
  • Prenatal period
  • Immediate postnatal period
  • Cost effectiveness
  • Prognostic value?

32
DR SCHUSTER
  • THANK YOU
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