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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.

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Cardiomyopathy the newborn l.jpg

CARDIOMYOPATHY&THE NEWBORN

N. Felicia Ochei, M.D.

Pediatrics-PL 2

November 2002


Introduction l.jpg
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 l.jpg
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*


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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*


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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


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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


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    Peripartum Cardiomyopathy

    The pediatrician’s Role

    • Liaison with OB

    • Careful maternal history

    • Anticipate problems from

      • Preterm delivery

      • Maternal Medications

      • Fetal distress

      • Instrumental delivery



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    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 vesselsor 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 l.jpg

    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:Pathophysiologic Classification


    Neonatal cardiomyopathy etiologic classification l.jpg

    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: Etiologic classification


    Neonatal cardiomyopathy clinical features l.jpg

    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:Clinical Features


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    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


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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

    • 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 l.jpg
    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 l.jpg
    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 l.jpg
    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


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    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 l.jpg
    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 l.jpg
    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 l.jpg

    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;Diagnostic Evaluation


    Neonatal cardiomyopathy management l.jpg

    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:Management


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    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)



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    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 review26 l.jpg
    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 review27 l.jpg
    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 review28 l.jpg
    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 review29 l.jpg
    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 review30 l.jpg
    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


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    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?


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    DR SCHUSTER

    THANK YOU


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