Related Experiment Videos
Factors affecting mortality in Ebstein's anomaly of the tricuspid valve
1Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
Insights
Ebstein's anomaly, a rare congenital heart defect, often presents with murmurs and cyanosis in infants. Younger age at diagnosis and specific echocardiographic features significantly increase the risk of cardiac death.
Area of Science:
- Pediatric Cardiology
- Congenital Heart Disease
- Cardiac Surgery
Background:
- Ebstein's anomaly is a rare congenital heart defect affecting the tricuspid valve.
- Clinical presentation varies widely, often diagnosed in infancy.
Purpose of the Study:
- To review clinical characteristics of Ebstein's anomaly.
- To identify factors influencing patient outcomes and survival.
Main Methods:
- Retrospective review of 21 patients with Ebstein's anomaly.
- Clinical data, ECG, chest X-ray, and echocardiography were analyzed.
- Follow-up ranged from 3 to 72 months.
Main Results:
- Common findings included systolic murmur (85.7%) and cyanosis (57.1%).
- Cardiomegaly (95.2%) and right bundle branch block (76.2%) were frequent.
- Surgical intervention was required in 28.6%, with a high mortality rate (5/6).
- Factors predicting cardiac death included younger age, infantile cyanosis, associated defects, leaflet displacement, and altered atrial/ventricular ratios.
Conclusions:
- Ebstein's anomaly poses significant risks, particularly in neonates and infants.
- Early diagnosis and identification of adverse prognostic factors are crucial for management.
- Surgical outcomes require careful consideration due to high mortality.
Abstract:
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 PA, 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.