CARDIOMYOPATHY & THE NEWBORN

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*

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*

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%)

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

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: 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

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 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 birth does not rule out disease in later life Avoid diuretics & inotropics Ventricular septal myomectomy Cardiac transplantation Those 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 1/3rd die; 1/3rd significant damage; 1/3rd 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:

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

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 time of presentation for the others

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

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)

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

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

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