Right ventricular long axis function in adults and children with Ebstein's malformation.

OBJECTIVES: To assess right ventricular function in adults and children with Ebstein's anomaly.
DESIGN: Prospective study.
SETTING: Tertiary referral centre.
SUBJECTS: Fifteen patients (8 adults and 7 children) with Ebstein's anomaly and 14 healthy controls.
INTERVENTIONS: Transthoracic echocardiography was performed in all. Right ventricular function was assessed from long axis M-mode recordings of the right atrio-ventricular free wall. Total systolic excursion as well as peak shortening and lenghtening rates of the right ventricle were measured.
RESULTS: Children and adult patients with Ebstein's anomaly differed in terms of age at diagnosis, the adult group having been diagnosed later 19.8+/-15.8 vs. 5.9+/-6.2 years, P<0.05. Measures of right ventricular long axis function in children with Ebstein's anomaly showed a significantly reduced systolic excursion 1.4+/-0.4 vs. 2.4+/-0.53 cm, P<0.05 and peak lenghtening rate; early 8.04+/-4.3 vs. 11.8+/-2.4 cm/s and late 6.14+/-3.6 vs. 10.6+/-4.3 cm/s, P=0.05 compared to controls. In contrast, measurements of right ventricular long axis function in adults with Ebstein's anomaly showed no significant difference when compared to the control group. Transtricuspid Doppler flow velocities were not different between patient's groups and corresponding controls.
CONCLUSION: The right ventricle assessed by this simple, non-invasive technique reveals a significantly reduced systolic and diastolic function in children with Ebstein's malformation compared to controls but a 'normal' right ventricular function comparable to controls in adult patients. Significant right ventricular dysfunction in children with Ebstein's anomaly could account for their earlier presentation. Long term follow up of the right ventricular abnormalities is needed in such children to discover more about the natural history of the disease.