Assessment of Left and Right Ventricular Function in Patients With Ebstein Anomaly With Different Echocardiographic Methods

Study Overview
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Brief Summary
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Ebstein anomaly is a rare congenital heart disease. It encompasses abnormalities of the tricuspid valve, the right atrium and the right ventricle (RV). Currently, cardiac magnetic resonance imaging (MRI) is considered the method of choice for the assessment of RV volume and function. To evaluate the right heart function VentriPoint method was designed to assess the RV in a fraction of the time, resulting in better efficacy and lower costs. VentriPoint is approved for Tetralogy of Fallot patients as well as patients with systemic RV in d-transposition of the great arteries. This method uses 2 dimensional standard ultrasound views linked to a VentriPoint Medical Systems unit. After acquisition, the physician identifies anatomical landmarks with dots on a number of the 2D ultrasound views through the heart. With a sensor (magnetic tracking system: GPS) on the ultrasound probe we know where the 2D plane is in 3D space. In this way the precise anatomical landmark is located in 3D space. The aim of the present study is to assess the feasibility, reliability and accuracy of the echocardiographic knowledge-based 3D reconstruction method to measure RV volume and function in patients with Ebstein anomaly. The accuracy of the method will be assessed by comparison with measurements obtained by cardiac MRI. In addition to the right ventricle the investigators want to assess the left ventricular function in patients with Ebstein anomaly in this study. By using speckle tracking echocardiography the investigators would like to investigate on the left ventricular contractility, rotation and synchrony of the left ventricle together with the RV. Not seldom the left ventricle is neglected in a patient with Ebstein anomaly by impressive findings of the right ventricle. Changes in the myocardial left ventricular structure (non compaction) are also described and may have negative impact on the function. The investigators want to analyze 25-30 patients with Ebstein anomaly including children starting at 11 years of age.

Official Title
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Left Ventricular Function in Ebstein Anomaly: Assessment by 3D Echocardiography and Speckle Tracking. Assessment and Validation of the Right Ventricle by VentriPoint Method.

Conditions
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Ebstein Anomaly

Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Ebstein anomaly male and female 11 years to 80 years of age Unoperated or operated on the heart, including 1 1/2 chamber palliation Wolff-Parkinson-White Syndrome Exclusion Criteria: moderate or severe mitral valve insufficiency, moderate or severe aortic insufficiency, moderate or severe aortic stenosis Univentricular palliation (Fontan) St.n. myectomy of the left ventricle Concomitant L-transposition of the great arteries

Ages Eligible for Study
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Minimum Age: 11 Years
Maximum Age: 80 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Ebstein anomaly<br>Patients with Ebstein anomaly | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Right ventricular volume (ml/m2) and function by VentriPoint. Structural myocardial changes in the left ventricle (Strain). | Comparison of the VentriPoint findings with measurements obtained by cardiac MRI. Structural changes of the left ventricle by echocardiography as well as ventricular longitudinal deformation in strain value by speckle tracking echocardiography. | 1 year | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Correlation of findings with clinical symptoms | Correlation with the severity of tricuspid regurgitation, the clinical symptoms, size and function of the right ventricle | 1 year |