Tricuspid cleft or tetracuspid valve? Usefulness of three-dimensional echocardiogram in the assessment of isolated tricuspid regurgitation in pediatrics.

Tricuspid regurgitation (TR) in children may be secondary to congenital anomalies of the tricuspid valve complex which is composed by annulus, leaflets, commissures, chordae tendineae, and papillary muscles. The most common congenital cause is Ebstein's anomaly; however, there are less frequent causes such as abnormal number of tricuspid leaflets, tricuspid cleft, leaflet prolapse, double orifice tricuspid valve, and congenital tricuspid valve dysplasia. Identifying the precise cause is important to plan an appropriate repair surgery. In this article, the case of a 4-year-old patient with a tetracuspid valve with significant tricuspid regurgitation is presented and the morphological analysis was made by two-dimensional (2D) and three-dimensional (3D) transthoracic echocardiography. The morphological differences between a tetracuspid valve and a cleft of the anterior leaflet tricuspid valve are exposed. 3D echocardiographic evaluation of the tricuspid valve allowed a better understanding of the tricuspid valve anatomy, which includes evaluation of the tricuspid annulus, leaflets, commissures, and subvalvular apparatus. Recognizing the accurate cause of isolated tricuspid regurgitation allows better planning of the surgical technique.

Assessment of Left Ventricular Myocardial Fibrosis in Adult Patients With Ebstein Anomaly: A Retrospective Cohort Study Based on Cardiac Magnetic Resonance and Histopathological Samples.

BACKGROUND: The association between Ebstein anomaly and myocardial fibrosis, particularly in the left ventricle, has been controversial. We aimed to assess the prevalence of replacement fibrosis with a focus on the left ventricle (LV) using cardiac magnetic resonance (CMR), make a histopathological association between LV fibrosis and CMR findings, and explore whether LV fibrosis is an independent risk factor for cardiovascular disease mortality using a derived risk score. METHODS: We performed a 12-year (2009-2021) retrospective cohort of adult patients with Ebstein anomaly who underwent CMR. The CMR evaluation included a comprehensive assessment of myocardial fibrosis by late gadolinium enhancement (LGE). Four postmortem samples were obtained from our cohort and stained using Masson trichrome to characterize LV fibrosis. We used Cox-regression analysis to identify and derive a prediction score that associated LV fibrosis with cardiovascular disease mortality. RESULTS: We included 57 adults with Ebstein anomaly (52% men; median age, 29.52 [interquartile range, 21.24-39.17] years), of whom 12 died during follow-up. LGE prevalence by CMR was observed in 52.6% in any chamber; LV-LGE in 29.8%. Histopathological findings revealed a mid-wall pattern with predominantly interstitial fibrosis and minimal replacement fibrosis. LV-LGE was associated with increased risk of cardiovascular disease mortality (hazard ratio, 6.02 [95% CI, 1.22-19.91]) attributable to lateral and mid-wall LV segment involvement. Our mortality score achieved an overall good prediction capacity (R2, 0.435; C statistic, 0.93; Dxy, 0.86). CONCLUSIONS: There is a high prevalence of LV fibrosis replacement in adults with Ebstein anomaly, characterized by specific CMR and histological patterns. Furthermore, LV-LGE fibrosis is an independent predictor of cardiovascular disease mortality, which could be integrated into risk assessment in clinical management.

Ebstein's anomaly: epidemiological analysis and presentation of different prenatal management.

OBJECTIVES: To present the crucial role of echocardiographic examination in perinatal care and analyze influence of prenatal treatment for neonatal outcome. Furthermore, the attempt to answer the question if there was any relationship between the occurrence of fetal Ebstein's anomaly and environmental risk factors in polish population. METHODS: Forty-five prenatal diagnoses of Ebstein's anomaly were compiled over the 21-year period (1998‒2018) form our single unit. The analysis included the assessment of maternal parameters (age, past obstetric history, and place of residence) and fetal parameters (sex, gestational age, anatomy, the fetal cardiovascular condition assessed by the CVPS, associated extracardiac anomalies or malformations, prenatal treatment, delivery and follow-up). RESULTS: The average age of gravida was 29.5 years (± 5.2 years) and gravidae <35 years of age accounted for 80% . There were 43 singleton pregnancies and 2 cases of multiple pregnancy. Ebstein's anomaly was mostly (averagely) diagnosed at 28th week of gestation. Forty-three fetuses had normal karyotypes and two had trisomy 21. Cardiomegaly was present in 91% (41) of fetuses. The average heart area to chest area ratio was 0.56 (± 0.12). In 21 cases, there was only fetal monitoring - echocardiographic examinations and postnatal mortality was 44.4%. In 5 cases, transplacental digoxin treatment was administered and mortality was: 40%. In another 5 cases, only steroid therapy was applied and postnatal mortality was 100%. Steroids and transplacental digoxin treatment were administered in 11 cases and mortality was 63.6%. In 3 last cases transplacental digoxin treatment, steroids and maternal hyperoxygenation therapy were given and mortality was 0%. Cesarean section rate was 49%. Moreover, due to Ebstein's anomaly regional peak of occurrence benzopyrene was deliberated as environmental risk factor. CONCLUSIONS: Fetal Ebstein's anomaly occurred in our population in healthy young women, expecting their first child and malformation was not related to fetus gender, nor to maternal health condition. Our data can be a new signal for the development of novel treatment strategies in therapy in fetuses with Ebstein's anomaly.

Assessment of left ventricular deformation in patients with Ebstein's anomaly by cardiac magnetic resonance tissue tracking.

PURPOSE: The aim of this study was to clarify the feasibility of myocardial strain using cardiovascular magnetic resonance (CMR) for the evaluation of left ventricular (LV) deformation in patients with Ebstein's anomaly (EA). MATERIALS AND METHODS: We recruited 32 patients with EA and 30 controls for CMR examination and measured LV function, dimension and tissue tracking parameters (the global and regional radial, circumferential and longitudinal peak strain), together with the right ventricle (RV) dimension. LV strain parameters were compared among the controls, patients with preserved LV ejection fraction (LVEF; ≥55%), and patients with reduced LVEF (<55%). Pearson's correlation was used to evaluate relationships between tissue tracking parameters with the RVEDD/LVEDD index and LVEF. An ROC analysis was also performed to determine whether the cut-off values for PS could be used to differentiate LV dysfunction between patients with EA and controls. The intraclass correlation coefficient (ICC) was used to assess the inter- and intra-observer variability. RESULTS: The global strain parameters all decreased significantly in the EA group compared with the control group (all P<0.05). Furthermore, the global radial and circumferential peak strain (PS) were obviously even lower in the reduced LVEF group than the strain measured in preserved LVEF groups (28.64% vs. 37.39%, p<0.05; and -8.20% vs. -17.89%; p<0.05; respectively). The regional strain abnormalities in EA patients were mainly involved in basal and middle segments. The results also demonstrated a significant correlation between the ratio of the RV end-diastolic dimension to the LV end-diastolic dimension (RVEDD/LVEDD index) with the global circumferential PS (r=0.508) and the longitudinal PS (r=0.474), as well as a good correlation between radial PS and LVEF (r=0.465). The ICCs for intra- and inter-observer variability were 0.797-0.904 and 0.701-0.896. CONCLUSIONS: LV strain serves an earlier and more comprehensive measurement of LV dysfunction than LVEF in EA, which could potentially be included as a supplementary diagnostic procedure in the evaluation of EA.

Non-volumetric echocardiographic indices and qualitative assessment of right ventricular systolic function in Ebstein's anomaly: comparison with CMR-derived ejection fraction in 49 patients.

AIMS: Ebstein's anomaly (EA) is often associated with right ventricular (RV) dysfunction. Data on echocardiographic quantification of RV function are, however, rare. The aim of this study was to determine how non-volumetric echocardiographic indices and qualitative assessment of global systolic RV function correlate with cardiovascular magnetic resonance (CMR)-derived RV ejection fraction (EF). METHODS AND RESULTS: We compared six echocardiographic indices and qualitative assessment of RV function with the gold standard CMR. A total of 49 unoperated patients with EA and a mean age of 32 ± 18 years were examined. Tricuspid annular plane systolic excursion, tissue Doppler myocardial velocities (peak S and IVA) and 2D strain and strain rate measures for the RV were compared with CMR-derived EF. Only 2D global longitudinal strain (2D-GLS), out of the six parameters investigated, showed a weak, although statistically significant correlation with CMR-derived RVEF (R = -0.4, P = 0.01). Using a cut-off value of -20.15, 2D-GLS sensitivity (77%) and specificity (46%) in detecting patients with a CMR-derived EF of <50% were comparable with qualitative assessment (sensitivity 77%, specificity 45%). CONCLUSION: Overall echocardiographic parameters of RV function correlate poorly with CMR-derived EF in patients with EA. Only 2D global longitudinal RV strain correlated weakly with CMR-derived RVEF. However, the sensitivity and specificity for detecting RV dysfunction using 2D strain imaging were comparable with qualitative RV functional assessment.

[Isolated left ventricular non-compaction associated with Ebstein's anomaly. Multimodality non-invasive imaging for the assessment of congenital heart disease]. / Miocardiopatía no compactada asociada a anomalía de Ebstein. Importancia de la imagen cardiaca multimodal en las cardiopatías congénitas.

To establish the etiology of heart failure in patients with congenital heart disease can be challenging. Multiple concomitant anomalies that can be missed after an initial diagnosis could be seen in these patients. In patients with congenital heart disease, a more accurate evaluation of cardiac morphology and left ventricular systolic function could be evaluated by recent non-invasive cardiac imaging techniques. We present a rare case where multimodal cardiac imaging was useful to establish the final diagnosis of left ventricular non-compaction associated with Ebstein's anomaly.