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Journal of the American College of... Aug 2023There are limited data on the prognostic role of right ventricular global longitudinal strain (RVGLS) in patients with Ebstein anomaly.
BACKGROUND
There are limited data on the prognostic role of right ventricular global longitudinal strain (RVGLS) in patients with Ebstein anomaly.
OBJECTIVES
This study sought to assess the relationship between RVGLS and mortality and to compare prognostic performance of RVGLS with conventional echocardiographic indices of right ventricular (RV) systolic function.
METHODS
This study identified adults with Ebstein anomaly with echocardiographic assessment of RV systolic function (RVGLS, RV fractional area change [RVFAC], RV tissue Doppler systolic velocity [RV s'], and tricuspid annular plane systolic excursion [TAPSE]) from 2003 to 2020. For ease of presentation, RVGLS was modeled as absolute values (ie, without the negative sign).
RESULTS
Of 620 patients (median age 37 years; men 261 [42%]), the mean absolute RVGLS, RVFAC, RV s', and TAPSE were 18% ± 5%, 32% ± 9%, 14 ± 6 cm/s, and 22 ± 8 mm, respectively. There were correlations between absolute RVGLS and RVFAC (r = 0.71; P < 0.001), between absolute RVGLS and RV s' (r = 0.41; P = 0.03), and between absolute RVGLS and TAPSE (r = 0.44; P = 0.002). Of 620 patients, 47 (8%) died during follow-up, and 34 of these deaths were cardiovascular. Absolute RVGLS was independently associated with all-cause mortality (adjusted HR: 0.94; 95% CI: 0.92-0.96 per unit increase) and cardiovascular mortality (adjusted HR: 0.92; 95% CI: 0.90-0.94 per unit increase). Absolute RVGLS had superior prognostic power (ie, ability to predict mortality) as compared with RVFAC, RV s', or TAPSE.
CONCLUSIONS
These data support the use of RVGLS for risk stratification in Ebstein anomaly, and further studies are required to assess how interventions may affect different patients according to risk stratification.
Topics: Male; Adult; Humans; Prognosis; Ebstein Anomaly; Global Longitudinal Strain; Echocardiography; Systole; Ventricular Function, Right; Ventricular Dysfunction, Right
PubMed: 37532420
DOI: 10.1016/j.jacc.2023.05.045 -
The Journal of Thoracic and... Jul 2024
Topics: Humans; Ebstein Anomaly; Infant, Newborn; Cardiac Surgical Procedures; Treatment Outcome; Heart Ventricles
PubMed: 37926199
DOI: 10.1016/j.jtcvs.2023.11.001 -
The Journal of Thoracic and... Dec 2023
Topics: Infant, Newborn; Humans; Ebstein Anomaly; Cardiac Surgical Procedures
PubMed: 37690623
DOI: 10.1016/j.jtcvs.2023.08.054 -
Journal of Clinical Ultrasound : JCU Apr 2024To analyze the influence of RV dysfunction evaluated by Free-angle M-mode (FAM) TAPSE Z-score on retrograde ductus arteriosus flow (RDAF) in fetuses with Ebstein anomaly...
Study on the correlation between retrograde ductus arteriosus flow and right ventricular function evaluated by Z-score of tricuspid annular plane systolic excursion in fetuses with Ebstein anomaly.
PURPOSE
To analyze the influence of RV dysfunction evaluated by Free-angle M-mode (FAM) TAPSE Z-score on retrograde ductus arteriosus flow (RDAF) in fetuses with Ebstein anomaly (EA).
METHODS
A retrospective cohort study of 30 EA and 60 normal fetuses were enrolled. The EA group was divided into two groups: with RDAF (EA-RDAF group) and without RDAF (EA-NRDAF group). FAM was used to measure TAPSE of EA and normal fetuses, and Z-scores were calculated. The differences of FAM-TAPSE Z-score, gestational week (GW), maternal age (MA), and mitral valve-tricuspid valve distance (MTD) between three groups were compared. The correlation and binary logistic regression between FAM-TAPSE Z-score, GW, MA, MTD, and RDAF were analyzed.
RESULTS
FAM-TAPSE Z-score was significantly lower in EA-RDAF group compared to other groups (p < 0.05). FAM-TAPSE Z-score, GW, and MA were negatively correlated with RDAF (p < 0.05), but no correlation was found between TR, MDT, and RDAF (p > 0.05). Multivariate logistic regression showed that FAM-TAPSE Z-score was an independent influencing factor for RDAF (OR = 0.102, p < 0.05).
CONCLUSION
RV dysfunction is an independent factor leading to RDAF in EA fetus, which provides a feasible theoretical basis for further study on improvement of RV function through intrauterine treatment to delay and prevent the RDAF, to avoid death cycle and improve live-birth rate.
PubMed: 38655706
DOI: 10.1002/jcu.23695 -
Rhode Island Medical Journal (2013) May 2024
Topics: Humans; Ebstein Anomaly; Echocardiography; Tricuspid Valve; Tricuspid Valve Insufficiency; Female; Adult
PubMed: 38687264
DOI: No ID Found -
The Journal of Thoracic and... Jul 2024
Topics: Humans; Ebstein Anomaly; Infant, Newborn; Cardiac Surgical Procedures; Treatment Outcome; Infant
PubMed: 37797933
DOI: 10.1016/j.jtcvs.2023.09.063 -
Ultrasound in Obstetrics & Gynecology :... Nov 2023To investigate the national prevalence and prenatal detection rate (DR) of major congenital heart disease (mCHD) in twin pregnancies without twin-to-twin transfusion...
OBJECTIVE
To investigate the national prevalence and prenatal detection rate (DR) of major congenital heart disease (mCHD) in twin pregnancies without twin-to-twin transfusion syndrome (TTTS)-associated CHD in a Danish population following a standardized prenatal screening program.
METHODS
This was a national registry-based study of data collected prospectively over a 10-year period. In Denmark, all women with a twin pregnancy are offered standardized screening and surveillance programs in addition to first- and second-trimester screening for aneuploidies and malformation, respectively: monochorionic (MC) twins every 2 weeks from gestational week 15 and dichorionic (DC) twins every 4 weeks from week 18. The data were retrieved from the Danish Fetal Medicine Database and included all twin pregnancies from 2009-2018, in which at least one fetus had a pre- and/or postnatal mCHD diagnosis. mCHD was defined as CHD requiring surgery within the first year of life, excluding ventricular septal defects. All pregnancy data were pre- and postnatally validated in the local patient files at the four tertiary centers covering the entire country.
RESULTS
A total of 60 cases from 59 twin pregnancies were included. The prevalence of mCHD was 4.6 (95% CI, 3.5-6.0) per 1000 twin pregnancies (1.9 (95% CI, 1.3-2.5) per 1000 live births). The prevalences for DC and MC were 3.6 (95% CI, 2.6-5.0) and 9.2 (95% CI, 5.8-13.7) per 1000 twin pregnancies, respectively. The national prenatal DR of mCHD in twin pregnancies for the entire period was 68.3%. The highest DRs were in cases with univentricular hearts (100%) and the lowest with aortopulmonary window, total anomalous pulmonary venous return, Ebstein's anomaly, aortic valve stenosis and coarctation of the aorta (0-25%). Mothers of children with prenatally undetected mCHD had a significantly higher body mass index (BMI) compared to mothers of children with a prenatally detected mCHD (median, 27 kg/m and 23 kg/m , respectively; P = 0.02).
CONCLUSIONS
The prevalence of mCHD in twins was 4.6 per 1000 pregnancies and was higher in MC than DC pregnancies. The prenatal DR of mCHD in twin pregnancies was 68.3%. Maternal BMI was higher in cases of prenatally undetected mCHD. © 2023 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.
Topics: Pregnancy; Child; Humans; Female; Pregnancy, Twin; Prevalence; Gestational Age; Heart Defects, Congenital; Twins, Dizygotic; Denmark; Retrospective Studies
PubMed: 37191390
DOI: 10.1002/uog.26249 -
European Heart Journal. Case Reports Dec 2023Ebstein's anomaly occurs when there is an apical displacement of the tricuspid valve with septal and posterior valve leaflets tethering. This condition often occurs in...
BACKGROUND
Ebstein's anomaly occurs when there is an apical displacement of the tricuspid valve with septal and posterior valve leaflets tethering. This condition often occurs in association with other congenital, structural, or conduction system diseases, including intracardiac shunts, valvular lesions, arrhythmias, accessory conduction pathways, and first-degree atrioventricular (AV) block. We present for the first time a case of a patient with Ebstein's anomaly who presented with second-degree Mobitz II AV block and was successfully treated with conduction system pacing (CSP) due to her young age and the likelihood of a long-term high percentage of pacing.
CASE SUMMARY
We present a case of a 42-year-old lady with a background of complex congenital heart disease, including severe pulmonary stenosis, Ebstein anomaly, and atrial septal defect (ASD). She required complex surgical intervention, including tricuspid valve (TV) repair and subsequently replacement, ASD closure, and pulmonary balloon valvuloplasty. She presented to our hospital with symptomatic second-degree Mobitz II AV block (dizziness, shortness of breath, and exercise intolerance) and right bundle branch block (RBBB) on her baseline ECG. Her echocardiogram showed dilated right ventricle (RV) and left ventricle (LV) with low normal LV systolic function. Due to her young age and the likelihood of a long-term high percentage of RV pacing, we opted for CSP after a detailed discussion and patient consent. The distal HIS position is the preferred pacing strategy at our centre. We could not cross the TV with the standard Medtronic C315 HIS catheter, so we had to use the deflectable C304 HIS catheter. Mapping and pacing of the distal HIS bundle were achieved by Medtronic Selectsecure 3830, 69 cm lead. HIS bundle pacing led to the correction of both second-degree Mobitz II AV block and pre-existing RBBB. The implantation was uneventful, and the patient was discharged home the next day without any acute complications.
DISCUSSION
Distal HIS pacing is feasible in patients with surgically treated complex Ebstein anomaly and heart block. This approach can normalize the QRS complex with a high probability of preserving or improving LV function.
PubMed: 38046645
DOI: 10.1093/ehjcr/ytad531 -
Echocardiography (Mount Kisco, N.Y.) Apr 20243D virtual and physical models from ultrasound scan data allow a 3D spatial view of congenital heart anomalies, interactive discussion among a multidisciplinary team,...
3D virtual and physical models from ultrasound scan data allow a 3D spatial view of congenital heart anomalies, interactive discussion among a multidisciplinary team, and improved parental counseling. To the best of our knowledge, this is the first description of 3D physical and virtual models of a fetal Ebstein anomaly.
Topics: Pregnancy; Female; Humans; Ebstein Anomaly; Heart Defects, Congenital; Ultrasonography, Prenatal
PubMed: 38549419
DOI: 10.1111/echo.15806 -
Anatolian Journal of Cardiology Aug 2023
Topics: Humans; Ebstein Anomaly; Heart Ventricles; Cardiomegaly; Aortic Dissection; Heart Atria
PubMed: 37288847
DOI: 10.14744/AnatolJCardiol.2023.3274