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Right ventricular long axis function in adults and children with Ebstein's malformation
1Cardiac Department, Royal Brompton and Harefield Trust and National Heart and Lung Institute, Imperial College, London, UK.
International Journal of Cardiology
|June 8, 2000
Summary
Children with Ebstein's anomaly have significantly reduced right ventricular function, unlike adults with the condition. This dysfunction in pediatric patients may explain earlier disease presentation and warrants further long-term study.
Area of Science:
- Cardiology
- Pediatric Cardiology
- Medical Imaging
Background:
- Ebstein's anomaly is a rare congenital heart defect affecting the tricuspid valve.
- Right ventricular (RV) function is crucial in patients with Ebstein's anomaly.
- Understanding RV function differences between pediatric and adult patients is important for management.
Purpose of the Study:
- To evaluate and compare right ventricular (RV) function in adult and pediatric patients diagnosed with Ebstein's anomaly.
- To assess RV function using transthoracic echocardiography and M-mode recordings.
- To compare RV function in patients with healthy controls.
Main Methods:
- Prospective study conducted at a tertiary referral center.
- Involved 15 patients (8 adults, 7 children) with Ebstein's anomaly and 14 healthy controls.
- Transthoracic echocardiography assessed RV long-axis function, including systolic excursion and peak shortening/lengthening rates.
Main Results:
- Children with Ebstein's anomaly exhibited significantly reduced RV systolic excursion and peak lengthening rates compared to controls.
- Adult patients with Ebstein's anomaly showed no significant difference in RV long-axis function compared to controls.
- Transtricuspid Doppler flow velocities were similar between patient groups and controls.
Conclusions:
- Children with Ebstein's anomaly demonstrate significantly impaired RV systolic and diastolic function.
- Adult patients with Ebstein's anomaly present with RV function comparable to healthy individuals.
- Reduced RV function in pediatric Ebstein's anomaly may contribute to earlier diagnosis and necessitates long-term follow-up to understand disease progression.