Key result
Pediatric Ebstein anomaly is linked to less spherical ventricles, decreased mass, and reduced EF in ~34%.
Why the study?
Patients with Ebstein anomaly have diverse clinical manifestations, and assessing structural and geometric characteristics of the heart is important for optimal management.
How do the structural and geometric characteristics of the heart differ in children with Ebstein anomaly compared to healthy controls?
Observational (n=2,153)
How do the structural and geometric characteristics of the heart differ in children with Ebstein anomaly compared to healthy controls?
Standard echocardiographic parameters like TAPSE and tissue Doppler may not accurately assess right ventricular contractility in Ebstein anomaly, whereas the myocardial performance index reflects decreased functional right ventricular volume.
Geometric changes in pediatric Ebstein anomaly may confound standard RV metrics; leaves open their prognostic utility and need for validation.
PURPOSE: Patients with Ebstein anomaly (EA) have a variety of clinical manifestation. The assessment of structural and geometric characteristics of the heart is important for optimal management. METHODS: We retrospectively analyzed echocardiography database from 2009 to 2020. We evaluate patients in two groups: patients with EA were in Group 1 and children without cardiovascular pathology were in Group 2. All children in both groups underwent echocardiography according to American Society of Echocardiography recommendations. The shape of the heart chambers and their function were studied in both groups. RESULTS: There were 153 in Group 1 and 2000 children without cardiovascular disease in Group 2. It was shown that in children with EA, the shape of the ventricle became less spherical, which was accompanied by a decrease in myocardial mass, and the ejection fraction was reduced 34% of patients. The functional volume (non-atrialized part) of the right ventricle in patients with EA was reduced, and its contractility was preserved in 62% of cases. Preservation of the contractile properties of the right ventricle in most cases was associated with higher systolic pressure in its cavity. CONCLUSION: TAPSE, TESV, and the velocity of the annulus fibrous ring movement according to tissue dopplerography in patients with EA do not allow us to assess the contractility of the right ventricle. The myocardial performance index (MPI) characterizes a decrease in the functional volume of the right ventricle.
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Aa et al. (2022) conducted an observational in Ebstein anomaly (n=2,153). Ebstein anomaly vs. Children without cardiovascular disease was evaluated on Shape and function of the heart chambers. Ebstein anomaly in children was associated with less spherical ventricles, decreased myocardial mass, and reduced ejection fraction in 34% of patients.
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