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Ebstein’s anomaly. Stephanie Merhar January 21, 2011. Anatomy. Pathophysiology. Associated defects. Commonly associated with: ASD or PFO (90%) VSD, AV canal defect Pulmonary stenosis/atresia (20-25%) Wolff-Parkinson-White Syndromes: Down, Marfan, Noonan, Cornelia de Lange

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ebstein s anomaly

Ebstein’s anomaly

Stephanie Merhar

January 21, 2011

associated defects
Associated defects
  • Commonly associated with:
    • ASD or PFO (90%)
    • VSD, AV canal defect
    • Pulmonary stenosis/atresia (20-25%)
    • Wolff-Parkinson-White
  • Syndromes:
    • Down, Marfan, Noonan, Cornelia de Lange
  • Maternal lithium ingestion?
  • Ebstein’s anomaly occurs in 0.3-0.8% of all congenital heart diseases
  • 1 in 20,000-50,000 live births
  • Equal male:female occurrence
  • Mortality in children presenting in the neonatal period is 30-50%
  • Mortality at all ages is 12.5%
prenatal presentation
Prenatal presentation
  • Difficult to diagnose prenatally
  • Fetal presentation is variable: possible features include cardiomegaly, RA enlargement, tricuspid regurgitation or dysplasia, arrhythmia, or fetal hydrops
  • Prognosis for the fetus diagnosed in utero with significant tricuspid valve disease is very poor (20% survival)
    • Progressive right heart dilatation
    • Cardiac failure
    • Lung hypoplasia
    • Pulmonary stenosis/atresia
neonatal presentation
Neonatal presentation
  • Congestive heart failure
    • Due to TR and RV dysfunction
  • Cyanosis
    • Decreased pulmonary blood flow due to R  L shunt through ASD or PFO
    • Increased pulmonary vascular resistance in the neonatal period compounds this problem
  • Murmur
physical exam
Physical exam
  • Heart sounds
    • First heart sound widely split with loud tricuspid component
    • Second heart sound usually is normal but may be widely split due to RBBB
    • Third and fourth heart sounds commonly present
  • Murmurs
    • Holosystolic murmur of tricuspid regurgitation
later presentation
Later presentation
  • Cyanosis
    • Due to R  L shunt at atrial level
  • Fatigue and dyspnea
    • Secondary to RV failure and decreased LV ejection fraction
  • Palpitations and sudden cardiac death
  • Incidental murmur
  • Paradoxic embolism
  • Due to right atrial enlargement and high prevalence of accessory pathways
  • 30-50% have evidence of WPW secondary to the atrialized RV tissue
  • Mapping and ablation are difficult
    • Atrial dilation disrupts anatomic landmarks
    • Accessory pathways are often multiple
initial management
Initial management
  • Prostaglandin infusion? (see next slide)
  • Placement of umbilical catheters
  • Initiation of mechanical ventilation
    • Minimum possible mean airway pressure
    • Tidal volumes of 10-15 ml/kg to overcome atelectasis
  • Management of pulmonary hypertension
management of pulmonary hypertension
Management of pulmonary hypertension
  • Nitric oxide
    • Reduces afterload of right ventricle
    • Helps distinguish functional from actual pulmonary atresia
  • Sedation
  • Other pulmonary vasodilators?
pges good or bad1
PGEs – good or bad?
  • Definitely need to start PGEs if functional pulmonary atresia
        • Need some way to get blood to lungs if going through PA is not an option
  • If patient gets worse on PGEs, discuss with cardiologist!
usual postnatal evaluation
Usual postnatal evaluation
  • Define anatomy with echocardiography
    • Nature of the RV outflow tract
      • If pulmonary atresia/severe stenosis, likelihood of biventricular repair is very low
    • Great Ormond Street Ebstein (GOSE) score for severity
      • Ratio of combined areas of true RA plus atrialized RV to the combined areas of the functional RV, LA, and LV
transport issues
Transport issues
  • Things to tell receiving cardiologist:
    • location of pulse ox (ideally pre and post), arterial blood gas, 4 point blood pressures, appearance of the CXR
  • Main problem to anticipate on transport
    • Desaturation!
    • Manage these babies like other babies with pulmonary hypertension
  • Aggarwal S, Chintala K, and R Humes. Sildenafil use in a symptomatic neonate with severe Ebstein’s anomaly. Am J Perinatol 2008; 25(2): 125-128.
  • Brown ML and JA Dearani. Ebstein malformation of the tricuspid valve: current concepts in management and outcomes. Curr Treat Options in CV Med 2009; 11:396-402.
  • Cherry C, Debord S, and N Moustapha-Nadler. Ebstein’s anomaly: a complex congenital heart defect. AORN Journal 2009; 89:1098-1111.
  • Dearani JA, O’Leary PW, and GK Danielson. Surgical treatment of Ebstein’s malformation: state of the art in 2006. Cardiol Youn 2006; 16(12-20).
  • Jaquiss RDB and M Imamura. Management of Ebstein’s anomaly and pure tricuspid insufficiency in the neonate. Semin Thorac Cardiovas Surg 2007;19:258-263.
  • Knott-Craig CJ and SP Goldbert. Management of neonatal Ebstein’s anomaly . Semin Thorac CV Surg 2007; 10:112-116.
  • Paranon S and P Acar. Ebstein’s anomaly of the tricuspid valve: from fetus to adult. Heart 2008; 94:237-243.
  • Pashia SE. Ebstein’s anomaly. Neonatal Network 2007; 26:197-207.