Download Ebstein`s anomaly

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GY
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06.2.26~
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06.2.20~
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Ebstein’s Anomaly
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유O구 F/29
Adm date: 06.02.20
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C/C: IUP 37+6 wks
fetal arrhythmia
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상기 산모 local OBGY에서 PN check받는 도중
NST상 fetal heart beat이 irregular하게 측정되
고 arrhythmia 소견 보여 본원으로 attending
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OB Hx
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P; 1-0-0-1
L.M.P.; 2005.5.30
E.D.C. ;2006.3.7
Family Hx: N-S
husband: ventricular arrythmia
Past Hx: N-S
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P/Ex
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Pv/Ex
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HOF: 36cm
Ut: 9 mo. Sized
Cx: closed
NST
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Ut cont: 3~4’, weak~mod
FHT: 60~180
USG
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Presentation: vertex
EFW: 3258g (53%)
CTAR: 31.46/68.12
Cardiomegaly
Enlarged RV due to TV regurgitation
R/O Ebstein’s anomaly
Operation
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C/sec (06.02.20)
Baby : F, 3445g , A/S 4->8, NICU
Definition
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Downward displacement of the septal
and posterior leaflets of the tricuspid
valve, with dysplasia of this valve
Etiology
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10% cases of chronic maternal lithium
intake during pregnancy
Associated Anomalies
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ASDS (secundum type or patent foramen
ovale)
Pulmonary atresia or stenosis
Patent ductus arteriosus
Tetralogy of Fallot
Coarctation of the aorta
Atrioventricular canal
Transposition of the great vessels
Pathology
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Two chambers within the right ventricle
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Atrialized portion: made up of parts of the
ventricular free wall, septum above the
functional opening of the tricuspid valve
infundibulum
Pathology
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Marked dilatatation of the “new” right atrial
cavity: regurgitant tricuspid jet, myocardial
contraction
Anterograde flow through the main pul. Artery:
severity of the tricuspid insufficiency,
functional capacity of the infundibular portion,
possible stenosis at the subpulmonary or
pulmonary valvular levels
Prenatal Life
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Difficult to access the permeability of the
outflow tract
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Entire right ventricular ejection fraction could go
backward through the leaking tricuspid valve,
which offers lower resistance then the pulmonary
valve
Severe tricuspid valve insufficiency, pulmonary
circulation will be essentially maintained by ductus
arteriosus, RL shunt through the foramen ovale is
increased
Fig. 4-41
Prognosis
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Prenatal prognosis could be
significantly influenced by the ability of
foramen ovale to decompress the right
atrium
Arrythmia due to extreme dilatation of
the RA might be a cause of sudden
intrauterine death
Obstetric Management
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Ebstein’s anomaly without cardiac
failure or hydrops: vaginal delivery
Cesarean section