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Ebstein' S Anomaly During Fetal Life:
Diagnosis and Perinatal Outcome

Leite, M.F.; Gianisella, R.B.; Zielinsky, P.

Fetal Cardiology Unit, Institute of Cardiology of
Rio Grande do Sul. Porto Alegre, Brasil

Background: Ebstein's anomaly is the most common congenital disease of the tricuspid valve, corresponding to 0,5% of congenital heart diseases. Mortality rate is reported to be in the 85% range in the neonatal period.
The purpose of this report is to review the experience of a tertiary center of Fetal Cardiology in the diagnosis and manegement of Ebstein's disease.

Material and Methods: Among 9500 fetal echocardiographic examinations from january 1987 once may 2000, 15 fetuses with Ebstein's anomaly were identified. Complete data of 12 of the patients were available for review.

Results: Mean gestational age at diagnosis was 31.4 weeks. No risk factors for cardiac diseases were present in 83%; 2 cases occurred in pregnacies at risk (lithium ingestion in one and maternal diabetes in the other). Fetal heart failure was present in the first examination in 5 fetuses. Atrial tachyarrhythmias were present in 2 patients, and were reverted to sinus rhythm with maternal digitalis. Fetal hydrops were observed in 2 cases. There were 4 deaths "in utero" and 3 in the neonatal period. All fetuses who died were in heart failure and the 3 neonates who died were in heart failure and the 3 neonates who did not survive had functional pulmonary atresia. Immediate neonatal surgery was performed in 3 babies, with 1 survival. The remaimimg patients are alive and well.

Conclusion: Ebstein's anomaly is a severe anomaly when detected during fetal life, and early prenatal diagnosis may contribute to optimize perinatal management.



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2do Congreso Virtual de Cardiología

Dr. Florencio Garófalo
Presidente Comité Organizador
Dr. Raúl Bretal
Presidente Comité Científico
Dr. Armando Pacher
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