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Cardiomyopathy And The Newborn
 

Cardiomyopathy And The Newborn

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    Cardiomyopathy And The Newborn Cardiomyopathy And The Newborn Presentation Transcript

    • CARDIOMYOPATHY & THE NEWBORN N. Felicia Ochei, M.D. Pediatrics-PL 2 November 2002
    • Introduction
      • Topics
      • Peripartum Cardiomyopathy: Implications to the fetal well-being
      • Review of Cardiomyopathy in the Neonatal period
      • Fetal Cardiomyopathy: A Journal article Review
    • 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*
    • 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*
    • 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%)
    • 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
    • Peripartum Cardiomyopathy
      • The pediatrician’s Role
      • Liaison with OB
      • Careful maternal history
      • Anticipate problems from
          • Preterm delivery
          • Maternal Medications
          • Fetal distress
          • Instrumental delivery
    • Neonatal Cardiomyopathy
    • 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
    • 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
    • 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)
    • 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
    • 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
    • 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
    • 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
    • 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
    • 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
    • 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
    • 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
    • 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
    • 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
    • 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
    • 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)
    • FETAL CARDIOMYOPATHY
    • 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
    • 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
    • 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
    • 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
    • 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)
    • 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
    • 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?
    • DR SCHUSTER THANK YOU