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Original ArticleOpen Access
Factors Affecting Mortality in Ebstein's Anomaly of the Tricuspid Valve
Khositseth A 
,
Khowsathit P
,
Khowsathit P Ebstein's anomaly of the tricuspid valve is a relatively uncommon congenital heart defect.
Twenty-one patients (11 boys and 10 girls) with Ebstein's anomaly were reviewed regarding
clinical characteristics and factors related to the outcome of this lesion. Their ages at presentation
ranged from 1 day to 13 years (median = 11 months). Eleven patients presented in infancy
period, 6 of them were neonates. Common clinical findings were systolic murmur (85.7%)
and cyanosis (57 .1% ). Laboratory findings included cardiomegaly on chest X-rays (95.2% ),
right bundle branch block pattern (76.2%) and right atrial enlargement (61.9%) on electrocardiography.
Diagnosis and grading of severity were established by echocardiography. Among
21 patients, 4 were lost during follow-up. Seventeen patients were followed for 3-72 months. Six
patients (28.6%) required surgery, 5 of whom died following surgery. Two patients died during
the medical follow-up. Factors affecting cardiac death were the younger age at presentation,
onset of cyanosis in infancy period, associated PS or P A, the lower insertion of the septal
leaflet of the tricuspid valve and the higher ratio of the combined area of right atrium and
atrialized right ventricle to that of functional right ventricle and left heart chambers.
Key word : Ebstein's Anomaly, Tricuspid Valve, Factor Affecting Mortality
Twenty-one patients (11 boys and 10 girls) with Ebstein's anomaly were reviewed regarding
clinical characteristics and factors related to the outcome of this lesion. Their ages at presentation
ranged from 1 day to 13 years (median = 11 months). Eleven patients presented in infancy
period, 6 of them were neonates. Common clinical findings were systolic murmur (85.7%)
and cyanosis (57 .1% ). Laboratory findings included cardiomegaly on chest X-rays (95.2% ),
right bundle branch block pattern (76.2%) and right atrial enlargement (61.9%) on electrocardiography.
Diagnosis and grading of severity were established by echocardiography. Among
21 patients, 4 were lost during follow-up. Seventeen patients were followed for 3-72 months. Six
patients (28.6%) required surgery, 5 of whom died following surgery. Two patients died during
the medical follow-up. Factors affecting cardiac death were the younger age at presentation,
onset of cyanosis in infancy period, associated PS or P A, the lower insertion of the septal
leaflet of the tricuspid valve and the higher ratio of the combined area of right atrium and
atrialized right ventricle to that of functional right ventricle and left heart chambers.
Key word : Ebstein's Anomaly, Tricuspid Valve, Factor Affecting Mortality
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