CARDIOMYOPATHY   &   THE NEWBORN N. Felicia Ochei, M.D. Pediatrics-PL 2 November 2002
Introduction <ul><li>Topics </li></ul><ul><li>Peripartum Cardiomyopathy: Implications to the fetal well-being </li></ul><u...
Peripartum Cardiomyopathy <ul><li>Definition </li></ul><ul><li>Dilated cardiomyopathy of uncertain origin characterized by...
Peripartum Cardiomyopathy <ul><li>Incidence </li></ul><ul><li>U.S.  1:1300 to 15,000 live births </li></ul><ul><li>Japan 1...
Peripartum Cardiomyopathy <ul><li>Medical Rx </li></ul><ul><ul><li>Inotropics </li></ul></ul><ul><ul><ul><li>Digioxin </li...
Peripartum Cardiomyopathy <ul><li>Fetal Implications </li></ul><ul><li>Fetal distress from maternal hypoxia </li></ul><ul>...
Peripartum Cardiomyopathy <ul><li>The pediatrician’s Role </li></ul><ul><li>Liaison with OB </li></ul><ul><li>Careful mate...
Neonatal Cardiomyopathy
Neonatal Cardiomyopathy <ul><li>Definitions </li></ul><ul><li>Neonate : B irth to 28 days of life </li></ul><ul><li>Neonat...
Neonatal Cardiomyopathy:   Pathophysiologic Classification <ul><li>WHO (1980)  </li></ul><ul><li>Guidance for therapy and ...
Neonatal Cardiomyopathy:  Etiologic classification <ul><li>DILATED </li></ul><ul><li>Perinatal insult/ maladjustment </li>...
Neonatal Cardiomyopathy: Clinical Features <ul><li>History </li></ul><ul><li>Non specific </li></ul><ul><li>Pallor, irrita...
Neonatal Cardiomyopathy: Asphyxia induced <ul><ul><li>Hypoxia leads to myocardial ischemia/dilation </li></ul></ul><ul><ul...
Neonatal Cardiomyopathy:   From Maternal Diabetes <ul><ul><li>Asymmetric hypertrophic cardiomyopathy </li></ul></ul><ul><u...
Neonatal Cardiomyopathy : Carnitine deficiency <ul><ul><li>Autosomal recessive inheritance </li></ul></ul><ul><ul><li>Plas...
Neonatal Cardiomyopathy: Myocarditis <ul><ul><li>Any infectious agent, commonly Coxsackie B, ECHO viruses, herpes, HIV, Ru...
Neonatal Cardiomyopathy : Pompe’s Disease <ul><ul><li>Generalized form of glycogen storage dse (type II) </li></ul></ul><u...
Neonatal Cardiomyopathy: 1diopathic Familial <ul><li>Multi gene disorder </li></ul><ul><li>Autosomal with variable penetra...
Neonatal Cardiomyopathy :   Endocardial Fibroelastosis <ul><ul><li>No established cause </li></ul></ul><ul><ul><li>Also ca...
Neonatal Cardiomyopathy : Anomalous origin of the left coronary artery <ul><li>From the pulmonary artery </li></ul><ul><li...
Neonatal Cardiomyopathy; Diagnostic Evaluation <ul><li>Step 1: Initial Evaluation </li></ul><ul><li>EKG </li></ul><ul><li>...
Neonatal Cardiomyopathy: Management <ul><li>Supportive Therapy </li></ul><ul><li>Non specific therapy for heart failure, t...
Neonatal Cardiomyopathy: Prognosis <ul><li>Not well described in infants  </li></ul><ul><li>Generally poor for infants </l...
FETAL CARDIOMYOPATHY
Fetal Cardiomyopathy :   A Journal Article Review <ul><li>  Schmidt KG, Einat B, Silverman NH, Scagneli SA. </li></ul><ul>...
Fetal Cardiomyopathy :   A Journal Article Review <ul><li>Study Objectives </li></ul><ul><li>To explore the possibility of...
Fetal Cardiomyopathy :   A Journal Article Review <ul><li>Study Methodology </li></ul><ul><li>625 women had fetal echocard...
Fetal Cardiomyopathy :   A Journal Article Review <ul><li>Study Findings </li></ul><ul><li>6 of  the 625 had dilated cardi...
Fetal Cardiomyopathy :   A Journal Article Review <ul><li>Study conclusions </li></ul><ul><li>Dilated cardiomyopathy may d...
Fetal Cardiomyopathy :   A Journal Article Review <ul><li>Study Limitations </li></ul><ul><li>Technical limitations: Unabl...
Fetal Cardiomyopathy:   A Journal Article Review <ul><li>Discussion </li></ul><ul><li>The value of fetal echocardiogram in...
DR SCHUSTER THANK YOU
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Cardiomyopathy And The Newborn

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Cardiomyopathy And The Newborn

  1. 1. CARDIOMYOPATHY & THE NEWBORN N. Felicia Ochei, M.D. Pediatrics-PL 2 November 2002
  2. 2. Introduction <ul><li>Topics </li></ul><ul><li>Peripartum Cardiomyopathy: Implications to the fetal well-being </li></ul><ul><li>Review of Cardiomyopathy in the Neonatal period </li></ul><ul><li>Fetal Cardiomyopathy: A Journal article Review </li></ul>
  3. 3. Peripartum Cardiomyopathy <ul><li>Definition </li></ul><ul><li>Dilated cardiomyopathy of uncertain origin characterized by: </li></ul><ul><li>Cardiac failure in the last month of pregnancy or within 5 months after delivery </li></ul><ul><li>Absence of demonstrable cause for the cardiac failure </li></ul><ul><li>Absence of demonstrable heart disease before the last month of pregnancy </li></ul><ul><li>Documented systolic dysfunction* </li></ul>
  4. 4. Peripartum Cardiomyopathy <ul><li>Incidence </li></ul><ul><li>U.S. 1:1300 to 15,000 live births </li></ul><ul><li>Japan 1:6000 live births </li></ul><ul><li>South Africa 1:1000 </li></ul><ul><li>Nigeria High incidence: ? related to tradition of ingestion dried lake salt </li></ul><ul><li>Age </li></ul><ul><li>Wide range probably more common > 30 years* </li></ul>
  5. 5. Peripartum Cardiomyopathy <ul><li>Medical Rx </li></ul><ul><ul><li>Inotropics </li></ul></ul><ul><ul><ul><li>Digioxin </li></ul></ul></ul><ul><ul><ul><li>Dobutamine when indicated </li></ul></ul></ul><ul><ul><li>Loop diuretics </li></ul></ul><ul><ul><li>Beta blockers </li></ul></ul><ul><ul><li>Anticoagulation </li></ul></ul><ul><ul><ul><li>Heparin(unfractionated, LMWH) </li></ul></ul></ul><ul><ul><ul><li>Warfarin (post partum) </li></ul></ul></ul><ul><ul><li>After load reduction </li></ul></ul><ul><ul><ul><li>Hydralazine </li></ul></ul></ul><ul><ul><ul><li>Nitrates </li></ul></ul></ul><ul><li>Obstetric mgt </li></ul><ul><ul><li>Spontaneous vaginal delivery at term is reasonable unless mother is decompensating </li></ul></ul><ul><ul><li>Painless and effortless labor/delivery </li></ul></ul><ul><ul><li>Inhaled analgesia preferred (epidural/spinal contraindicated for 24hrs after use of LMWH) </li></ul></ul><ul><ul><li>Forceps/vacuum assisted delivery is the rule </li></ul></ul><ul><ul><li>Vaginal delivery preferred as C/S carries a higher risk of PE and and endometritis (75%) </li></ul></ul>
  6. 6. Peripartum Cardiomyopathy <ul><li>Fetal Implications </li></ul><ul><li>Fetal distress from maternal hypoxia </li></ul><ul><li>Placental hypo-perfusion </li></ul><ul><ul><ul><li>Poor cardiac output </li></ul></ul></ul><ul><ul><ul><li>Excessive use of diuretics </li></ul></ul></ul><ul><ul><ul><li>Hypotension from afterload reducers </li></ul></ul></ul><ul><li>Complications of instrumental delivery </li></ul><ul><li>Complications of intra partum anesthesia (choice & quantity) </li></ul><ul><li>Risks of Preterm delivery </li></ul><ul><ul><ul><li>Severe maternal decompensation </li></ul></ul></ul><ul><li>Adverse effects of medications (e.g. Digoxin, Beta blockers, LMWH) </li></ul><ul><ul><ul><li>Safety for use in pregnancy not established </li></ul></ul></ul><ul><li>Psychosocial issues </li></ul><ul><ul><ul><li>Infant maternal bonding </li></ul></ul></ul>
  7. 7. Peripartum Cardiomyopathy <ul><li>The pediatrician’s Role </li></ul><ul><li>Liaison with OB </li></ul><ul><li>Careful maternal history </li></ul><ul><li>Anticipate problems from </li></ul><ul><ul><ul><li>Preterm delivery </li></ul></ul></ul><ul><ul><ul><li>Maternal Medications </li></ul></ul></ul><ul><ul><ul><li>Fetal distress </li></ul></ul></ul><ul><ul><ul><li>Instrumental delivery </li></ul></ul></ul>
  8. 8. Neonatal Cardiomyopathy
  9. 9. Neonatal Cardiomyopathy <ul><li>Definitions </li></ul><ul><li>Neonate : B irth to 28 days of life </li></ul><ul><li>Neonatal Cardiomyopathy : Disease of the neonate in which the myocardium is affected without primary abnormalities of the valves, great vessels or septum </li></ul><ul><li>Epidemiology </li></ul><ul><li>Difficult to define: Few studies, rare disease entities </li></ul><ul><li>Estimates: 1: 10,000 live births (Nelson) </li></ul><ul><li>Constitutes about 1% of childhood cardiac disease </li></ul><ul><li>10% of all pediatric cardiac deaths </li></ul>
  10. 10. Neonatal Cardiomyopathy: Pathophysiologic Classification <ul><li>WHO (1980) </li></ul><ul><li>Guidance for therapy and prognosis </li></ul><ul><li>Dilated Cardiomyopathy </li></ul><ul><ul><li>Insult to the myocardium </li></ul></ul><ul><ul><li>tissue necrosis/interstitial fibrosis </li></ul></ul><ul><ul><li>impaired systolic contractility/diastolic compliance </li></ul></ul><ul><ul><li>ventricular dilation to maintain function </li></ul></ul><ul><ul><li>Left +/- right sides </li></ul></ul><ul><li>Hypertrophic Cardiomyopathy </li></ul><ul><ul><li>Myocyte hypertrophy & disarray </li></ul></ul><ul><ul><li>Increased mass & thickness </li></ul></ul><ul><ul><li>Increased mass/volume ratio </li></ul></ul><ul><ul><li>Poor diastolic chamber compliance Left ventricle </li></ul></ul><ul><ul><li>High systolic pressure gradient </li></ul></ul><ul><li>Restrictive Cardiomyopathy </li></ul><ul><ul><li>Rare, very small L ventricular cavity </li></ul></ul><ul><ul><li>Impaired diastolic function initially </li></ul></ul><ul><li>Unclassified cardiomyopathy </li></ul>
  11. 11. Neonatal Cardiomyopathy: Etiologic classification <ul><li>DILATED </li></ul><ul><li>Perinatal insult/ maladjustment </li></ul><ul><ul><li>Asphyxia </li></ul></ul><ul><ul><li>Persistent fetal circulation </li></ul></ul><ul><li>Congenital anomalies </li></ul><ul><ul><li>Anomalous origin of Left coronary </li></ul></ul><ul><li>Inborn errors of metabolism </li></ul><ul><ul><li>Glycogen storage dses (Pompe’s dse) </li></ul></ul><ul><ul><li>Mucopolysaccharidosis </li></ul></ul><ul><ul><li>Disorders of fatty acid metabolism (Carnitine deficiency) </li></ul></ul><ul><ul><li>Amino & organic acidiurias </li></ul></ul><ul><li>Maternal connective Tissue dse </li></ul><ul><ul><li>SLE </li></ul></ul><ul><li>HYPERTROPHIC </li></ul><ul><li>Familial </li></ul><ul><ul><li>Idiopathic Hypertrophic </li></ul></ul><ul><li>Maternal disease </li></ul><ul><ul><li>Diabetes </li></ul></ul><ul><li>Myocarditis </li></ul><ul><ul><li>Infectious </li></ul></ul><ul><ul><li>endotoxins, exotoxicins </li></ul></ul><ul><li>Drugs /Iatrogenic </li></ul><ul><ul><li>Dexamathasone (BPD)( case report) </li></ul></ul><ul><ul><li>ECMO ( case report) </li></ul></ul><ul><ul><li>Adriamycin </li></ul></ul><ul><ul><li>Chloramphenicol </li></ul></ul><ul><li>Malformation syndromes </li></ul><ul><ul><li>Beckwith wiedemann </li></ul></ul><ul><ul><li>Noonan </li></ul></ul><ul><ul><li>Leopard </li></ul></ul><ul><ul><li>Downs (case report) </li></ul></ul>
  12. 12. Neonatal Cardiomyopathy: Clinical Features <ul><li>History </li></ul><ul><li>Non specific </li></ul><ul><li>Pallor, irritability </li></ul><ul><li>Tachypnea </li></ul><ul><li>Diaphoresis </li></ul><ul><li>Fatigue esp with feeds </li></ul><ul><li>Poor wt gain </li></ul><ul><li>PE </li></ul><ul><li>Signs of CCF: </li></ul><ul><ul><li>Tachypnea, tachycardia, narrow pulse p </li></ul></ul><ul><ul><li>Decreased peripheral pulse, hepatomegaly, wheezing </li></ul></ul><ul><ul><li>+/- cyanosis </li></ul></ul><ul><ul><li>Murmur of mitral insufficiency </li></ul></ul><ul><ul><li>+/- left ventricular outflow obstruction(hypertrophic) </li></ul></ul><ul><li>Features of underlying etiology </li></ul><ul><li>EKG </li></ul><ul><ul><li>Flat T wave </li></ul></ul><ul><ul><li>ST depression </li></ul></ul><ul><ul><li>Generalized low voltages </li></ul></ul><ul><ul><li>Characteristic findings for the underlying abnormality </li></ul></ul><ul><li>CXR </li></ul><ul><ul><li>Cardiomegaly </li></ul></ul><ul><ul><li>May be normal in fulminant cases </li></ul></ul><ul><ul><li>Pulmonary edema </li></ul></ul><ul><ul><li>Pericardial effusion may be present (Water-bottle configuration) </li></ul></ul><ul><li>ECHO </li></ul><ul><ul><li>Diagnostic </li></ul></ul><ul><ul><li>Ventricular dilatation/dyskinesia </li></ul></ul><ul><ul><li>Ventricular outflow obstruction </li></ul></ul>
  13. 13. Neonatal Cardiomyopathy: Asphyxia induced <ul><ul><li>Hypoxia leads to myocardial ischemia/dilation </li></ul></ul><ul><ul><li>Term infant with delivery complicated by hypoxic stress </li></ul></ul><ul><ul><li>Apgars usually <3 @ 1 </li></ul></ul><ul><ul><li>Metabolic acidosis/ multi system ischemia </li></ul></ul><ul><ul><li>Severe cases: Hypotension/shock </li></ul></ul><ul><ul><li>Murmur of mitral/tricuspid regurg may be present </li></ul></ul><ul><ul><li>EKG: Diffuse ST -T changes, R atrial hypertrophy </li></ul></ul><ul><ul><li>Prognosis: Good without cardiogenic shock </li></ul></ul>
  14. 14. Neonatal Cardiomyopathy: From Maternal Diabetes <ul><ul><li>Asymmetric hypertrophic cardiomyopathy </li></ul></ul><ul><ul><li>Mechanism not clearly understood ? Hyperinsulinemia </li></ul></ul><ul><ul><li>Prevalence unrelated to diabetic control of mother </li></ul></ul><ul><ul><li>Puffy, Plethoric infant, with signs and symptoms of CCF </li></ul></ul><ul><ul><li>SEM common and related to degree of outflow obstruction </li></ul></ul><ul><ul><li>RX:Usually symptomatic </li></ul></ul><ul><ul><li>Prognosis: Usually good, resolves in months </li></ul></ul><ul><ul><li>Digitalis and other inotropics agents are contraindicated </li></ul></ul><ul><ul><li>except in very severe depression of myocardial contractility </li></ul></ul>
  15. 15. Neonatal Cardiomyopathy : Carnitine deficiency <ul><ul><li>Autosomal recessive inheritance </li></ul></ul><ul><ul><li>Plasma memb carnitine transport defect: Impairs fatty acid oxidation </li></ul></ul><ul><ul><li>Metabolic acidosis, intractable hypoglycemia, severe non-immune hydrops, +/-muscle weakness </li></ul></ul><ul><ul><li>EKG: Giant T waves(pathognomonic) </li></ul></ul><ul><ul><li>Subnormal carnitine level 1-2 %, heterozygous parents have 50 % levels </li></ul></ul><ul><ul><li>Symptomatic Rx for the cardiac failure gives minimal benefits </li></ul></ul><ul><ul><li>Definitive Rx: Oral carnitine supplements </li></ul></ul><ul><ul><li>Prognosis: Usually good with early diagnosis and Rx </li></ul></ul><ul><ul><li>Risk of growth and mental retardation </li></ul></ul>
  16. 16. Neonatal Cardiomyopathy: Myocarditis <ul><ul><li>Any infectious agent, commonly Coxsackie B, ECHO viruses, herpes, HIV, Rubella </li></ul></ul><ul><ul><li>Bacterial/fungal infections </li></ul></ul><ul><ul><li>Vertical/horizontal spread </li></ul></ul><ul><ul><li>Pathology: multicellular infiltrates </li></ul></ul><ul><ul><li>Usually first 10 days of life </li></ul></ul><ul><ul><li>Features of acute infective process </li></ul></ul><ul><ul><li>Involvement of other organs like CNS esp Coxsackie B </li></ul></ul><ul><ul><li>Gamma globulins beneficial </li></ul></ul><ul><ul><li>Rx underlying infection: Interferon, Ribavirin </li></ul></ul>
  17. 17. Neonatal Cardiomyopathy : Pompe’s Disease <ul><ul><li>Generalized form of glycogen storage dse (type II) </li></ul></ul><ul><ul><li>Lysosomal alpha- glucosidase deficiency </li></ul></ul><ul><ul><li>Autosomal recessive </li></ul></ul><ul><ul><li>Infiltrative cardiomyopathy </li></ul></ul><ul><ul><li>Skeletal muscular hypotonia: Protruding tongue, feeble cry, poor feeding </li></ul></ul><ul><ul><li>Hyporeflexia </li></ul></ul><ul><ul><li>Diagnosis: Measurement of enzyme activity or DNA analysis </li></ul></ul><ul><ul><li>EKG: (characteristic) </li></ul></ul><ul><ul><ul><li>Short PR interval </li></ul></ul></ul><ul><ul><ul><li>prominent P waves </li></ul></ul></ul><ul><ul><ul><li>massive QRS voltage </li></ul></ul></ul><ul><ul><li>Uniformly fatal </li></ul></ul>
  18. 18. Neonatal Cardiomyopathy: 1diopathic Familial <ul><li>Multi gene disorder </li></ul><ul><li>Autosomal with variable penetrance </li></ul><ul><li>Ventricular dysrhthmias/ Sudden death </li></ul><ul><li>Normal Echo @ birth does not rule out disease in later life </li></ul><ul><li>Avoid diuretics & inotropics </li></ul><ul><li>Ventricular septal myomectomy </li></ul><ul><li>Cardiac transplantation </li></ul><ul><li>Those presenting @ birth have worse prognosis </li></ul>
  19. 19. Neonatal Cardiomyopathy : Endocardial Fibroelastosis <ul><ul><li>No established cause </li></ul></ul><ul><ul><li>Also called elastic tissue hyperplasia </li></ul></ul><ul><ul><li>Pathology: White opaque fibroblastic thickening of the endocardium </li></ul></ul><ul><ul><li>1:6000 (1960); 1:70,000 (1980) </li></ul></ul><ul><ul><li>Infants < 6 months usually </li></ul></ul><ul><ul><li>Severe CCF/ rhythm disturbances </li></ul></ul><ul><ul><li>Failure to thrive </li></ul></ul><ul><ul><li>CXR : Massive cardiomegaly </li></ul></ul><ul><ul><li>EKG: Low voltage as in severe myocarditis </li></ul></ul><ul><ul><li>ECHO: Bright -appearing endocardial surface </li></ul></ul>
  20. 20. Neonatal Cardiomyopathy : Anomalous origin of the left coronary artery <ul><li>From the pulmonary artery </li></ul><ul><li>Should be ruled out in all cases of cardiomyopathy </li></ul><ul><li>EKG: anterolateral infarct </li></ul><ul><li>Surgical correction usually successful </li></ul>
  21. 21. Neonatal Cardiomyopathy; Diagnostic Evaluation <ul><li>Step 1: Initial Evaluation </li></ul><ul><li>EKG </li></ul><ul><li>CXR </li></ul><ul><li>ECHO </li></ul><ul><li>Step 2: Screening Evaluation </li></ul><ul><li>CBC </li></ul><ul><li>CMP </li></ul><ul><li>Enzymes:LDH, SGOT, SGPT, CPK, aldolase </li></ul><ul><li>ABG </li></ul><ul><li>Fractionated serum carnitine </li></ul><ul><li>Urine organic & amino acids </li></ul><ul><li>Urine muco/oligosacharides </li></ul><ul><li>Skeletal survey </li></ul><ul><li>Viral studies: Stool, NPW, urine, blood </li></ul><ul><li>Step 3: Specific Testing </li></ul><ul><li>Cardiac catheterization </li></ul><ul><li>Myocardial biopsy </li></ul><ul><li>Holter monitoring </li></ul><ul><li>Carnitine levels (skeletal, cardiac tissue, urine) </li></ul><ul><li>Serum ketone bodies, ammonia, pyruvate, lactate </li></ul><ul><li>Fibroblast studies </li></ul><ul><li>Chromosomes </li></ul>
  22. 22. Neonatal Cardiomyopathy: Management <ul><li>Supportive Therapy </li></ul><ul><li>Non specific therapy for heart failure, to improve survival & alleviate symptoms </li></ul><ul><li>ACE inhibitors (captopril, enalpril) </li></ul><ul><ul><li>Reduce afterload </li></ul></ul><ul><ul><li>Improve cardiac ejection </li></ul></ul><ul><ul><li>Reduce catecholamine drive prolonging cardiac survival </li></ul></ul><ul><ul><li>Careful titration necessary </li></ul></ul><ul><li>B blockers (metoprolol, carvedilol) </li></ul><ul><li>Digoxin </li></ul><ul><li>Diuretics </li></ul><ul><li>Specific Therapy </li></ul><ul><li>Depends on the underlying disease condition </li></ul><ul><ul><li>Most have no effective Rx </li></ul></ul><ul><ul><li>Carnitine supplements </li></ul></ul><ul><ul><li>Surgery </li></ul></ul><ul><ul><ul><li>Correction of aberrant vessels </li></ul></ul></ul><ul><ul><ul><li>Implanable defibrillators </li></ul></ul></ul><ul><ul><ul><li>Partial left venticulectomy </li></ul></ul></ul><ul><ul><ul><li>Cardiac transplant </li></ul></ul></ul>
  23. 23. Neonatal Cardiomyopathy: Prognosis <ul><li>Not well described in infants </li></ul><ul><li>Generally poor for infants </li></ul><ul><li>Depends on underlying condition </li></ul><ul><li>Some carry 100% mortality rate e.g. Pompe,s disease </li></ul><ul><li>Annual mortality 6% -8% in children </li></ul><ul><li>One year survival rate: 63% </li></ul><ul><li>5 year survival rate </li></ul><ul><li>Clinical adage 1/3 rd die; 1/3 rd significant damage; 1/3 rd recover (infective myocarditis) </li></ul>
  24. 24. FETAL CARDIOMYOPATHY
  25. 25. Fetal Cardiomyopathy : A Journal Article Review <ul><li> Schmidt KG, Einat B, Silverman NH, Scagneli SA. </li></ul><ul><li> </li></ul><ul><li>Echocardiographic Evaluation of Dilated Cardiomyopathy in the Human Fetus </li></ul><ul><li>The American Journal of Cardiology 1989; 63:599-605 </li></ul>
  26. 26. Fetal Cardiomyopathy : A Journal Article Review <ul><li>Study Objectives </li></ul><ul><li>To explore the possibility of detecting dilated cardiomyopathy in the prenatal period </li></ul><ul><li>To follow the the development of the disease during gestation </li></ul><ul><li>To determine the effects of prenatal presentation on the postnatal course of the disease </li></ul>
  27. 27. Fetal Cardiomyopathy : A Journal Article Review <ul><li>Study Methodology </li></ul><ul><li>625 women had fetal echocardiography at the Univ. of California in San Francisco from 1980 to 1987 </li></ul><ul><li>Criteria for inclusion in the study: </li></ul><ul><ul><li>Family history of congenital heart defects </li></ul></ul><ul><ul><li>Abnormal findings in obstetrics sonogram </li></ul></ul><ul><ul><li>No history of antecedent maternal illness </li></ul></ul><ul><li>The echo was performed from 20 to 26 weeks gestation for family Hx and @ time of presentation for the others </li></ul>
  28. 28. Fetal Cardiomyopathy : A Journal Article Review <ul><li>Study Findings </li></ul><ul><li>6 of the 625 had dilated cardiomyopathy but had structurally normal hearts </li></ul><ul><li>2 fetuses referred for family Hx had normal findings initially but later developed cardiomyopathy on serial ECHOs </li></ul><ul><li>Abnormal findings included: </li></ul><ul><ul><li>Reduced systolic myocardial performance(5) </li></ul></ul><ul><ul><li>AV valve regurgitation (3) </li></ul></ul><ul><ul><li>Abnormal chamber dimensions (3) </li></ul></ul><ul><li>4 deaths (1 fetus, 3 neonates) 1 survivor required cardiac transplant in infancy </li></ul>
  29. 29. Fetal Cardiomyopathy : A Journal Article Review <ul><li>Study conclusions </li></ul><ul><li>Dilated cardiomyopathy may develop during fetal life </li></ul><ul><li>Diagnosis can be achieved by serial echocardiogram </li></ul><ul><li>Normal findings in mid-trimester do not always rule out the subsequent development of cardiomyopathy </li></ul><ul><li>Reduced systolic performance; most sensitive finding and preceded the presence of progressive dilation </li></ul><ul><li>Fetal onset cardiomyopathy carried poor prognosis </li></ul><ul><ul><li>(Conflicts with other studies that suggested better outcomes for early childhood onset) </li></ul></ul><ul><li>There were no predictive factors for outcome of the disease </li></ul><ul><li> ( Similar to findings in studies of dilated cardiomyopathy in childhood) </li></ul>
  30. 30. Fetal Cardiomyopathy : A Journal Article Review <ul><li>Study Limitations </li></ul><ul><li>Technical limitations: Unable to calculate ventricular volumes ejection fraction earlier in the study </li></ul><ul><li>Difficulty comparing chamber enlargements and performance with normal values </li></ul><ul><ul><li>As was with all previous studies </li></ul></ul><ul><ul><li>No defined normal values </li></ul></ul>
  31. 31. Fetal Cardiomyopathy: A Journal Article Review <ul><li>Discussion </li></ul><ul><li>The value of fetal echocardiogram in cardiomyopathy </li></ul><ul><ul><li>Research and further development </li></ul></ul><ul><ul><li>Fetal echo usually done not solely for cardiomyopathy but for cardiac anomalies in general </li></ul></ul><ul><ul><li>Intervention ? </li></ul></ul><ul><ul><ul><li>Prenatal period </li></ul></ul></ul><ul><ul><ul><li>Immediate postnatal period </li></ul></ul></ul><ul><ul><li>Cost effectiveness </li></ul></ul><ul><ul><li>Prognostic value? </li></ul></ul>
  32. 32. DR SCHUSTER THANK YOU
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