CARDIOMYOPATHY
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CARDIOMYOPATHY Presentation Transcript

  • 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. 1:1300 to 15,000 live births
    • Japan 1:6000 live births
    • South Africa 1:1000
    • Nigeria High incidence: ? related to tradition of ingestion dried lake salt
    • Age
    • Wide range probably more common > 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
    • Psychosocial issues
        • Infant maternal bonding
  • 7. Peripartum Cardiomyopathy
    • The pediatrician’s 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 : B irth 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
    • DILATED
    • Perinatal insult/ maladjustment
      • Asphyxia
      • Persistent fetal circulation
    • Congenital anomalies
      • Anomalous origin of Left coronary
    • Inborn errors of metabolism
      • Glycogen storage dses (Pompe’s dse)
      • Mucopolysaccharidosis
      • Disorders of fatty acid metabolism (Carnitine deficiency)
      • Amino & organic acidiurias
    • Maternal connective Tissue dse
      • SLE
    • 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
      • Noonan
      • Leopard
      • Downs (case report)
  • 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(hypertrophic)
    • 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 <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
  • 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
      • RX:Usually 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 : 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
  • 18. Neonatal Cardiomyopathy: 1diopathic Familial
    • Multi gene disorder
    • Autosomal with variable penetrance
    • Ventricular dysrhthmias/ Sudden death
    • Normal Echo @ birth does not rule out disease in later life
    • Avoid diuretics & inotropics
    • Ventricular septal myomectomy
    • Cardiac transplantation
    • Those presenting @ 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
      • 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
  • 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
    • Enzymes:LDH, 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 1/3 rd die; 1/3 rd significant damage; 1/3 rd 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; 63:599-605
  • 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 @ 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
    • ( Similar to findings in studies of dilated cardiomyopathy in childhood)
  • 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