Pediatric Ebstein Anomaly Clinical Presentation

Updated: Aug 14, 2018
  • Author: Duraisamy Balaguru, MBBS, MRCP, FACC, FAAP, FSCAI; Chief Editor: Stuart Berger, MD  more...
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Presentation

History

For the purpose of clinical presentation of Ebstein anomaly, the patients are separated into the age groups, as used in a study by Celermajer et al. [10]

Fetus

An abnormal fetal scan is present in about 86% of fetuses, and an arrhythmia is present in about 5%. [11]

Neonate (aged 0-1 mo)

About 74% of neonates have cyanosis, 10% have heart failure with poor feeding and failure to thrive, and 9% have an incidental heart murmur.

Infant (aged 2 mo to 2 y)

About 35% of infants have cyanosis, 43% have heart failure with poor feeding and failure to thrive, and 13% have an incidental heart murmur.

Child (aged 3-10 y)

About 14% of children have cyanosis, 8% have heart failure with poor growth and decreased exercise tolerance, 12% have an arrhythmia with complaints of palpitations, and 66% have an incidental heart murmur.

Adolescent (aged 11-18 y)

About 13% of adolescents have cyanosis, 13% have heart failure with dyspnea on exertion and decreased exercise tolerance, 40% have an arrhythmia with complaints of palpitations, and 33% have an incidental heart murmur.

Adult (aged >18 y)

About 4% of adults have cyanosis, 26% have heart failure with dyspnea on exertion and decreased exercise tolerance, 43% have an arrhythmia with complaints of palpitations, 13% have an incidental heart murmur, 20% have chest pain, and 6% have syncope.

Next:

Physical Examination

The physical examination findings in patients with Ebstein anomaly vary based on the age of the patient and the degree of tricuspid valve regurgitation and right ventricular outflow tract obstruction. Note the following:

  • The classic cardiac examination is marked by a gallop or quadruple rhythm caused by widely split first and second heart sounds, as well as a third or fourth heart sound.

  • Tricuspid regurgitation causes a holosystolic or regurgitant systolic murmur at the left lower sternal border in the newborn and during infancy. The murmur is a shorter, systolic murmur in older children and adults due to low velocity flow from the hypokinetic, low pressure in the right ventricle.

  • A diastolic murmur may be heard secondary to increased flow through a normal-sized or stenotic tricuspid valve orifice. A systolic ejection murmur associated with right ventricular outflow tract obstruction may also be heard.

  • Congestive heart failure, if present, may cause passive liver congestion, and the liver edge may be easily palpable below the right costal margin.

  • Elevated jugular venous distention with prominent "V" wave may be present in older patients. Clubbing may be seen in a few patients who have had persistent cyanosis.

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