8/10/2019 Ebstein Anomalie
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"nvasive diagnostic testing is not as commonly perormed today as
in the
past due to the availability o better noninvasive techniues.
,ertain
patients $ith Ebstein's anomaly# ho$ever# may reuire cardiac
catheteri1ationto ully deine their cardiac anatomy and
unction.
Some patients $ith abnormal heart rhythms may reuire
electrophysiologic testing to better identiy and potentially
treat their
heart rhythm problems.
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How is Ebstein's anomaly treated?
3our child's pediatric cardiologist $ill discuss the treatment
options
appropriate or your child. &ild deects may reuire no speciic
treatment#
only prophylais or bacterial endocarditis. &edical treatment
is used to
help children $ith congestive heart ailure or abnormal heart
rhythms.
Surgery may be indicated# depending on a child's speciic
circumstances.
Surgical repair or replacement o the tricuspid valve and closure
o the
ormamen ovale or atrial septal deect may be recommended in
older
children $ith moderate to severe congestive heart ailure#
signiicant heart
enlargement# cyanosis# or abnormal clot ormation.
Abnormal accessory conduction path$ays that allo$ ast heart
rates(supraventricular tachycardia! to develop may be ablated
(removed! using
procedures in the catheteri1ation laboratory.
&edical therapy or heart ailure or arrhythmias is typically
used in
con0unction $ith planned surgical intervention. "n very severe
orms o
Ebstein's anomaly# an operation may be reuired in the ne$born
period
and the treatment strategy is more li%e that or children $ith
single
ventricle cardiac anomalies.
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What are the results of treatment?
Surgical outcomes $ere reported in a study o 456 patients $ith
Ebstein's
anomaly $ho had surgical repair or replacement o the
tricuspid
valve. 2ver hal o these patients had valve repair# $hile 78
percent had
valve replacement. T$elve patients (8 percent! died $ithin the
irst
postoperative month. Ten died ater this irst month. 9eart ailure
status
signiicantly improved in 67 percent o the 4:: surviving
patients.
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