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Hussain Dhanani - One of the best experts on this subject based on the ideXlab platform.

Rebecca S. Beroukhim - One of the best experts on this subject based on the ideXlab platform.

  • effect of in utero non steroidal anti inflammatory drug therapy for severe Ebstein Anomaly or tricuspid valve dysplasia nsaid therapy for fetal Ebstein Anomaly
    American Journal of Cardiology, 2021
    Co-Authors: Lindsay R Freud, Rebecca S. Beroukhim, Louise Wilkinshaug, Terra Lafranchi, Colin K Phoon, Julie S Glickstein, Kristopher M Cumbermack, Majd Makhoul, Shaine A Morris, Heather Y Sun
    Abstract:

    Ebstein Anomaly (EA) and tricuspid valve dysplasia (TVD) are rare congenital malformations associated with nearly 50% mortality when diagnosed in utero. The diseases often produce severe tricuspid regurgitation (TR) in the fetus and in some cases, pulmonary regurgitation (PR) and circular shunting ensue. Since the ductus arteriosus (DA) plays a critical role in the circular shunt and may be constricted by transplacental nonsteroidal anti-inflammatory drugs (NSAIDs), we sought to assess the effect of NSAIDs on fetuses with EA/TVD. We reviewed mothers of singleton fetuses with EA/TVD and PR, indicative of circular shunting, who were offered NSAIDs at multiple centers from 2010 to 2018. Initial dosing consisted of indomethacin, followed by ibuprofen in most cases. Twenty-one patients at 10 centers were offered therapy at a median gestational age (GA) of 30.0 weeks (range: 20.9 to 34.9). Most (15/21 = 71%) mothers received NSAIDs, and 12 of 15 (80%) achieved DA constriction after a median of 2.0 days (1.0 to 6.0). All fetuses with DA constriction had improved PR; 92% had improved Doppler patterns. Median GA at pregnancy outcome (live-birth or fetal demise) was 36.1 weeks (30.7 to 39.0) in fetuses with DA constriction versus 33 weeks (23.3 to 37.3) in fetuses who did not receive NSAIDs or achieve DA constriction (p = 0.040). Eleven of 12 patients (92%) with DA constriction survived to live-birth, whereas 4 of 9 patients (44%) who did not receive NSAIDs or achieve DA constriction survived (p = 0.046). In conclusion, our findings demonstrate the proof of concept that NSAIDs mitigate circular shunt physiology by DA constriction and improve PR among fetuses with severe EA/TVD. Although the early results are encouraging, further investigation is necessary to determine safety and efficacy.

  • impact of the cone operation on left ventricular size function and dyssynchrony in Ebstein Anomaly a cardiovascular magnetic resonance study
    Journal of Cardiovascular Magnetic Resonance, 2018
    Co-Authors: Rebecca S. Beroukhim, Linyuan Jing, David M. Harrild, Brandon K. Fornwalt, Jonathan Rhodes, Abba Mejiaspiegeler, Sitaram Emani
    Abstract:

    In addition to tricuspid regurgitation (TR) and right ventricular (RV) enlargement, patients with Ebstein Anomaly are at risk for left ventricular (LV) dysfunction and dyssynchrony. We studied the impact of the cone tricuspid valve reconstruction operation on LV size, function, and dyssynchrony. All Ebstein Anomaly patients who had both pre- and postoperative cardiovascular magnetic resonance (CMR) studies were retrospectively identified. From cine images, RV and LV volumes and ejection fractions (EF) were calculated, and LV circumferential and longitudinal strain were measured by feature tracking. To quantify LV dyssynchrony, temporal offsets (TOs) were computed among segmental circumferential strain versus time curves using cross-correlation analysis and patient-specific reference curves. An LV dyssynchrony index was calculated as the standard deviation of the TOs. Twenty patients (65% female) were included with a median age at cone operation of 16 years, and a median time between pre- and postoperative CMR of 2.8 years. Postoperatively, there was a decline in the TR fraction (56 ± 19% vs. 5 ± 4%, p < 0.001), RV end-diastolic volume (EDV) (242 ± 110 ml/m2 vs. 137 ± 82 ml/m2, p < 0.001), and RV stroke volume (SV) (101 ± 35 vs. 51 ± 7 ml/m2, p < 0.001). RV EF was unchanged. Conversely, there was an increase in both LV EDV (68 ± 13 vs. 85 ± 13 ml/m2, p < 0.001) and LV stroke volume (37 ± 8 vs. 48 ± 6 ml/m2, p < 0.001). There was no change in LV EF, or global circumferential and longitudinal strain but basal septal circumferential strain improved (16 ± 7% vs. 22 ± 5%, p = 0.04). LV contraction become more synchronous (dyssynchrony index: 32 ± 17 vs. 21 ± 9 msec, p = 0.02), and the extent correlated with the reduction in RV EDV and TR. In patients with the Ebstein Anomaly, the cone operation led to reduced TR and RV stroke volume, increased LV stroke volume, improved LV basal septal strain, and improved LV synchrony. Our data demonstrates that the detrimental effect of the RV on LV function can be mitigated by the cone operation.

  • Impact of the cone operation on left ventricular size, function, and dyssynchrony in Ebstein Anomaly: a cardiovascular magnetic resonance study
    'Springer Science and Business Media LLC', 2018
    Co-Authors: Rebecca S. Beroukhim, Linyuan Jing, David M. Harrild, Brandon K. Fornwalt, Abba Mejia-spiegeler, Jonathan Rhodes, Sitaram Emani, Andrew J. Powell
    Abstract:

    Abstract Background In addition to tricuspid regurgitation (TR) and right ventricular (RV) enlargement, patients with Ebstein Anomaly are at risk for left ventricular (LV) dysfunction and dyssynchrony. We studied the impact of the cone tricuspid valve reconstruction operation on LV size, function, and dyssynchrony. Methods All Ebstein Anomaly patients who had both pre- and postoperative cardiovascular magnetic resonance (CMR) studies were retrospectively identified. From cine images, RV and LV volumes and ejection fractions (EF) were calculated, and LV circumferential and longitudinal strain were measured by feature tracking. To quantify LV dyssynchrony, temporal offsets (TOs) were computed among segmental circumferential strain versus time curves using cross-correlation analysis and patient-specific reference curves. An LV dyssynchrony index was calculated as the standard deviation of the TOs. Results Twenty patients (65% female) were included with a median age at cone operation of 16 years, and a median time between pre- and postoperative CMR of 2.8 years. Postoperatively, there was a decline in the TR fraction (56 ± 19% vs. 5 ± 4%, p 

Candice K Silversides - One of the best experts on this subject based on the ideXlab platform.

  • right heart characteristics and exercise parameters in adults with Ebstein Anomaly new perspectives from cardiac magnetic resonance imaging studies
    International Journal of Cardiology, 2013
    Co-Authors: Daniel Tobler, Sergey Yalonetsky, Andrew M Crean, Candice K Silversides, John Granton, Luke J Burchill, Rachel M Wald
    Abstract:

    Abstract Background The utility of cardiac magnetic resonance imaging (CMR) for assessment of adults with Ebstein Anomaly is not well-defined. We sought to evaluate CMR characteristics in this population and to relate these to exercise parameters. Methods We analyzed CMR studies in adults with unrepaired Ebstein Anomaly for measures of severity of Ebstein disease, including atrialized, functional and total right ventricular (RV) volumes, ejection fraction (EF) and severity index (area of atrialized RV+right atrium/functional RV+left ventricle+left atrium). We related these CMR values to cardiopulmonary exercise test measurements. Results Twenty-seven adults (mean age 41±14years, 70% female) were included. Functional RV end-diastolic volume (EDV) was 150±68mL/m 2 and atrialized RVEDV was 25±24mL/m 2 . In 17 patients (63%), the functional RVEDV was enlarged (>114mL/m 2 ). Percent predicted peak VO 2 for the population was 65±20%. On univariable analysis, peak VO 2 was inversely related to atrialized RVEDV (p=0.011), total RVEDV (p=0.041), functional RVEDV/left ventricular EDV ratio (p=0.015) and magnitude of tricuspid valve displacement (p=0.031). In the multivariate model, the only CMR factor to relate to peak VO 2 was atrialized RVEDV (p=0.011, β=−0.48). No significant correlations were found between CMR measures and heart rate response or ventilatory response to exercise. Conclusion In adults with unrepaired Ebstein Anomaly, atrialized RV volume was independently related to aerobic capacity. The volume of the atrialized RV is a novel CMR measure which may express severity of disease. Further research is needed to evaluate the prognostic relevance of this exploratory work.

  • cardiac magnetic resonance imaging and the assessment of Ebstein Anomaly in adults
    American Journal of Cardiology, 2011
    Co-Authors: Sergey Yalonetsky, Daniel Tobler, Matthias Greutmann, Andrew M Crean, Bernd J Wintersperger, Elsie T Nguyen, Erwin Oechslin, Candice K Silversides, Rachel M Wald
    Abstract:

    No published studies have evaluated the role of cardiac magnetic resonance (CMR) imaging for the assessment of Ebstein Anomaly. Our objective was to evaluate the right heart characteristics in adults with unrepaired Ebstein Anomaly using contemporary CMR imaging techniques. Consecutive patients with unrepaired Ebstein Anomaly and complete CMR studies from 2004 to 2009 were identified (n = 32). Volumetric measurements were obtained from the short-axis and axial views, including assessment of the functional right ventricular (RV) end-diastolic volume (EDV) and end-systolic volume. The volume of the atrialized portion of the right ventricle in end-diastole was calculated as the difference between the total RVEDV and the functional RVEDV. The reproducibility of the measurements in the axial and short-axis views was determined within and between observers. The median value derived from the short-axis and axial views was 136 ml/m 2 (range 59 to 347) and 136 ml/m 2 (range 63 to 342) for the functional RVEDV, 153 ml/m 2 (range 64 to 441) and 154 ml/m 2 (range 67 to 436) for the total RVEDV, 49% (range 32% to 46%) and 50% (range 40% to 64%) for the functional RV ejection fraction, respectively. The axial measurements demonstrated lower intraobserver and interobserver variability than the short-axis approach for all values, with the exception of the intraobserver functional RVEDV and interobserver total RVEDV for which the limits of agreement and variance were not significantly different between the 2 views. In conclusion, measurements of right heart size and systolic function in patients with Ebstein Anomaly can be reliably achieved using CMR imaging. Axial imaging appeared to provide more reproducible data than that obtained from the short-axis views.

  • canadian cardiovascular society 2009 consensus conference on the management of adults with congenital heart disease outflow tract obstruction coarctation of the aorta tetralogy of fallot Ebstein Anomaly and marfan s syndrome
    Canadian Journal of Cardiology, 2010
    Co-Authors: Candice K Silversides, Barbara J.m. Mulder, Marla Kiess, Luc M Beauchesne, Timothy J Bradley, Michael S Connelly, Koichiro Niwa, Gary D Webb, Jack M Colman, Judith Therrien
    Abstract:

    With advances in pediatric cardiology and cardiac surgery, the population of adults with congenital heart disease (CHD) has increased. In the current era, there are more adults with CHD than children. This population has many unique issues and needs. Since the 2001 Canadian Cardiovascular Society Consensus Conference report on the management of adults with CHD, there have been significant advances in the field of adult CHD. Therefore, new clinical guidelines have been written by Canadian adult CHD physicians in collaboration with an international panel of experts in the field. Part II of the guidelines includes recommendations for the care of patients with left ventricular outflow tract obstruction and bicuspid aortic valve disease, coarctation of the aorta, right ventricular outflow tract obstruction, tetralogy of Fallot, Ebstein Anomaly and Marfan's syndrome. Topics addressed include genetics, clinical outcomes, recommended diagnostic workup, surgical and interventional options, treatment of arrhythmias, assessment of pregnancy risk and follow-up requirements. The complete document consists of four manuscripts that are published online in the present issue of The Canadian Journal of Cardiology. The complete document and references can also be found at www.ccs.ca or www.cachnet.org.

Matthias Greutmann - One of the best experts on this subject based on the ideXlab platform.

  • left ventricular non compaction prevalence in congenital heart disease
    International Journal of Cardiology, 2013
    Co-Authors: Barbara E Stahli, Sabine Klaassen, Catherine Gebhard, Patric Biaggi, Emanuela Valsangiacomo R Buechel, Christine Attenhofer Jost, Rolf Jenni, Felix C Tanner, Matthias Greutmann
    Abstract:

    INTRODUCTION: Left ventricular non-compaction cardiomyopathy (LVNC) is a rare cardiomyopathy, originally described as an isolated disease without other structural cardiac abnormalities. The aim of this study was to explore the prevalence of LVNC among adults with different types of congenital heart disease. METHODS: From our databases we identified adults with congenital heart disease who fulfilled diagnostic criteria for LVNC. We report frequencies of associated congenital cardiac defects and the prevalence of LVNC among patients with different congenital heart defects. RESULTS: From a total of 202 patients with LVNC, 24 patients (12%; mean age 32±11years, 19 males) had additional congenital cardiac defects. Associated defects were left ventricular outflow tract abnormalities in 11 patients (46%), including 7 uni- or bicuspid aortic valves; two aortic coarctations; one diffuse aortic hypoplasia and one subaortic stenosis, Ebstein Anomaly in 6 patients (25%), tetralogy of Fallot in two (8%), and double outlet right ventricle in one patient (4%). In our cohort, the prevalence of LVNC was highest among patients with Ebstein Anomaly (6/40, 15%), followed by aortic coarctation (2/60, 3%), tetralogy of Fallot (3/129, 2%) and uni- or bicuspid aortic valves (7/963, 1%). CONCLUSION: In adults, various forms of congenital heart disease are associated with LVNC, particularly stenotic lesions of the left ventricular outflow tract, Ebstein Anomaly, and tetralogy of Fallot. In the future, studying these patients in more depth may provide a better understanding of the interplay between genetic and hemodynamic factors that lead to the phenotype of LVNC.

  • cardiac magnetic resonance imaging and the assessment of Ebstein Anomaly in adults
    American Journal of Cardiology, 2011
    Co-Authors: Sergey Yalonetsky, Daniel Tobler, Matthias Greutmann, Andrew M Crean, Bernd J Wintersperger, Elsie T Nguyen, Erwin Oechslin, Candice K Silversides, Rachel M Wald
    Abstract:

    No published studies have evaluated the role of cardiac magnetic resonance (CMR) imaging for the assessment of Ebstein Anomaly. Our objective was to evaluate the right heart characteristics in adults with unrepaired Ebstein Anomaly using contemporary CMR imaging techniques. Consecutive patients with unrepaired Ebstein Anomaly and complete CMR studies from 2004 to 2009 were identified (n = 32). Volumetric measurements were obtained from the short-axis and axial views, including assessment of the functional right ventricular (RV) end-diastolic volume (EDV) and end-systolic volume. The volume of the atrialized portion of the right ventricle in end-diastole was calculated as the difference between the total RVEDV and the functional RVEDV. The reproducibility of the measurements in the axial and short-axis views was determined within and between observers. The median value derived from the short-axis and axial views was 136 ml/m 2 (range 59 to 347) and 136 ml/m 2 (range 63 to 342) for the functional RVEDV, 153 ml/m 2 (range 64 to 441) and 154 ml/m 2 (range 67 to 436) for the total RVEDV, 49% (range 32% to 46%) and 50% (range 40% to 64%) for the functional RV ejection fraction, respectively. The axial measurements demonstrated lower intraobserver and interobserver variability than the short-axis approach for all values, with the exception of the intraobserver functional RVEDV and interobserver total RVEDV for which the limits of agreement and variance were not significantly different between the 2 views. In conclusion, measurements of right heart size and systolic function in patients with Ebstein Anomaly can be reliably achieved using CMR imaging. Axial imaging appeared to provide more reproducible data than that obtained from the short-axis views.

Olivella Alberto - One of the best experts on this subject based on the ideXlab platform.

  • Anomalía de Ebstein asociada con el síndrome de cri du chat (llanto de gato) y duplicación 20q
    'Anales de la Facultad de Medicina', 2021
    Co-Authors: Olivella Alberto
    Abstract:

    La anomalía de Ebstein es una cardiopatía congénita de baja prevalencia y alta mortalidad en las primeras etapas de la vida. En la literatura médica, no se ha informado de una asociación entre la anomalía de Ebstein y el síndrome cri du chat. A continuación, presentamos el caso de un recién nacido a término con bajo peso para su edad y que tenía un diagnóstico prenatal de anomalía de Ebstein y un diagnóstico posnatal de síndrome cri du chat y duplicación 20q detectada en array CGH. El paciente requirió tratamiento médico con soporte inotrópico, ventilación de alta frecuencia y óxido nítrico, con adecuada respuesta. No fue necesaria la intervención quirúrgica.Ebstein Anomaly is a congenital heart defect with a low prevalence and high mortality in the early stages of life. In medical literature, there is no reported association between Ebstein Anomaly and cri du chat syndrome. Here, we report the case of a full-term newborn with a low weight for his age and who had a prenatal diagnosis of Ebstein Anomaly and a postnatal diagnosis of cri du chat syndrome and 20q duplication detected on array CGH. The patient required medical treatment with inotropic support, high-frequency ventilation and nitric oxide, with an adequate response. Surgical intervention was not needed

  • Anomalía de Ebstein asociada con síndrome de cri du chat (llanto de gato) y duplicación 20q
    'BMJ', 2020
    Co-Authors: Olivella Alberto, Manotas Hernan, Payán-gómez César, Piñeros, Juan Gabriel
    Abstract:

    Ebstein Anomaly is a congenital heart defect with a low prevalence and high mortality in the early stages of life. In medical literature, there is no reported association between Ebstein Anomaly and cri du chat syndrome. Here, we report the case of a full-term newborn with a low weight for his age and who had a prenatal diagnosis of Ebstein Anomaly and a postnatal diagnosis of cri du chat syndrome and 20q duplication detected on array CGH. The patient required medical treatment with inotropic support, high-frequency ventilation and nitric oxide, with an adequate response. Surgical intervention was not needed