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JTCVS Open Dec 2023
PubMed: 38204705
DOI: 10.1016/j.xjon.2023.09.002 -
JTCVS Open Dec 2023In this study, we investigated the clinical characteristics of patients with Ebstein anomaly and left ventricular outflow tract (LVOT) obstruction as well as possible...
OBJECTIVE
In this study, we investigated the clinical characteristics of patients with Ebstein anomaly and left ventricular outflow tract (LVOT) obstruction as well as possible mechanisms of obstruction as assessed by 2-dimensional echocardiography.
METHODS
We queried our institutional echocardiography and surgical databases for patients with the diagnosis of Ebstein anomaly seen from 1985 through 2022. Fourteen patients had the additional diagnosis of LVOT obstruction identified from transthoracic echocardiography.
RESULTS
The mean age of the 14 patients at the time that LVOT obstruction was identified was 51.9 ± 9.9 years, and 11 (78%) were female. LVOT obstruction (mean gradient 61.0 ± 25.7 mm Hg) was documented at the time of initial diagnosis of Ebstein anomaly for 8, and in the remaining, was recognized on follow-up imaging. Dynamic LVOT obstruction with systolic anterior motion (SAM) was the most common mechanism and was mostly accompanied by leftward septal movement and septal hypertrophy. Seven of the 9 patients with SAM had the diagnosis of hypertrophic cardiomyopathy. Leftward bowing of the septum appeared to be the sole cause of LVOT obstruction in 3 and a contributing factor to SAM in 4. Among the 13 patients who underwent an operation for Ebstein anomaly, 7 had concomitant septal myectomy and 3 had a subsequent procedure.
CONCLUSIONS
There are multiple mechanisms of LVOT obstruction in patients with Ebstein anomaly, including SAM and leftward bowing of the interventricular septum, which may exist alone or in combination. Septal myectomy at the time of tricuspid valvuloplasty is safe and effectively reduces gradients in the outflow tract and may prevent the need for subsequent reintervention.
PubMed: 38204684
DOI: 10.1016/j.xjon.2023.10.024 -
JTCVS Open Dec 2023To describe the surgical outcomes in neonates and infants who had surgery for Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD).
OBJECTIVE
To describe the surgical outcomes in neonates and infants who had surgery for Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD).
METHODS
Retrospective chart review for all patients who underwent surgery for EA or TVD during the index hospitalization after birth at our institution from January 2005 to February 2023.
RESULTS
Fifteen symptomatic neonates and infants who had surgery for EA or TVD were included, 8 with EA and 7 with TVD. Eleven patients (73%) and 3 patients (20%) required preoperative inotropes and extracorporeal membrane oxygenation, respectively. Nine patients (60%) had a Starnes procedure and 6 patients (40%) had tricuspid valve repair (TVr). Mortality at last follow-up was 27% overall (n = 4/15), 22% after Starnes (n = 2/9) and 33% after TVr (n = 2/6), without a significant difference despite a greater-risk profile in the Starnes group. Postoperative day 1 lactate level was associated with mortality on Cox regression (hazard ratio, 1.45; = .01). Three of 9 patients who had a Starnes procedure were or will be converted to a cone repair (1.5/2-ventricle repair).
CONCLUSIONS
Mortality after surgery for EA or TVD during the index hospitalization after birth is still significant in the current era and is associated with a greater lactate level at postoperative day 1. The Starnes procedure and TVr had comparable outcomes despite a greater-risk profile in the Starnes group. An initial single-ventricle approach does not preclude conversion to biventricular or 1.5-ventricle repair.
PubMed: 38204669
DOI: 10.1016/j.xjon.2023.08.007 -
Korean Circulation Journal Feb 2024We aimed to investigate long-term clinical and echocardiographic outcomes, including tricuspid valve durability, annular growth, and left ventricular reverse remodeling,...
BACKGROUND AND OBJECTIVE
We aimed to investigate long-term clinical and echocardiographic outcomes, including tricuspid valve durability, annular growth, and left ventricular reverse remodeling, after modified cone reconstruction in patients with Ebstein's anomaly.
METHODS
This was a retrospective analysis of all pediatric patients who underwent modified cone reconstruction for Ebstein's anomaly at a single tertiary center between January 2005 and June 2021.
RESULTS
A total of 14 pediatric patients underwent modified cone reconstruction for Ebstein's anomaly; the median age was 5.8 years (range, 0.01-16.6). There were three patients (21.4%) with Carpentier type B, ten patients with Carpentier type C (71.4%), and one patient with Carpentier type D (7.1%). There was no early or late mortality, arrhythmia, or readmission for heart failure at a 10-year follow-up. There were no cases of more than mild tricuspid stenosis or more than moderate tricuspid regurgitation during the study period, except for one patient with severe tricuspid regurgitation who underwent reoperation. The z value for tricuspid valve annular size significantly decreased immediately after the operation (2.46 vs. -1.15, p<0.001). However, from 1 year to 7 years after surgery, the z values were maintained between -1 and +1. Left ventricular end-systolic volume, end-diastolic volume, and stroke volume increased after surgery and remained elevated until seven years postoperatively.
CONCLUSIONS
Ebstein's anomaly in children can be repaired by modified cone reconstruction with low mortality and morbidity, good tricuspid valve durability, and annular growth relative to somatic growth.
PubMed: 38196117
DOI: 10.4070/kcj.2023.0200 -
Journal of the American Heart... Jan 2024The increasing prevalence of atrial fibrillation (AF) in adults with congenital heart disease raises significant questions regarding its management. The unique... (Review)
Review
The increasing prevalence of atrial fibrillation (AF) in adults with congenital heart disease raises significant questions regarding its management. The unique underlying anatomic and physiological background further adds to the difficulty in eliminating the AF burden in these patients. Herein, we provide an overview of the current knowledge on the pathophysiology and risk factors for AF in adult congenital heart disease, with a special focus on the existing challenges in AF ablation. Emerging imaging modalities and ablation techniques might have a role to play. Evidence regarding the safety and efficacy of AF ablation in adult congenital heart disease is summarized, especially for patients with an atrial septal defect, Ebstein anomaly of the tricuspid valve, tetralogy of Fallot, and Fontan circulation. Finally, any remaining gaps in knowledge and potential areas of future research are highlighted.
Topics: Humans; Adult; Heart Defects, Congenital; Atrial Fibrillation; Heart Septal Defects, Atrial; Catheter Ablation; Ebstein Anomaly
PubMed: 38193287
DOI: 10.1161/JAHA.123.032102 -
Kardiologia Polska 2023
Successful pregnancy course and outcome in a patient with unusual coincidence of two structural heart defects: Ebstein anomaly and biventricular non-compaction cardiomyopathy - extremely rare, but there!
Topics: Female; Pregnancy; Humans; Ebstein Anomaly; Cardiomyopathies; Heart Diseases; Patients
PubMed: 38189509
DOI: 10.33963/v.kp.98283 -
Indian Pacing and Electrophysiology... 2024Accessory pathway ablation in Ebstein anomaly can be significantly more challenging than in structurally normal hearts. An alternative to the conventional approach to...
Accessory pathway ablation in Ebstein anomaly can be significantly more challenging than in structurally normal hearts. An alternative to the conventional approach to mapping APs is to detect points with a high-density mapping catheter based on an automated detection algorithm using open window mapping. It detects the sharpest signal at each point with high-density mapping rather than relying on the origin of the local electrogram to localize the pathway and determine a site for successful ablation. We herein report the first case in literature of a redo-accessory pathway ablation in Ebstein anomaly using this technique.
PubMed: 38176468
DOI: 10.1016/j.ipej.2023.12.007 -
BMJ Case Reports Dec 2023Complete trisomy 5 is a rare and lethal abnormality. Mosaic trisomy 5 presents in various phenotypes, ranging from a clinically normal fetus to fetuses presenting...
Complete trisomy 5 is a rare and lethal abnormality. Mosaic trisomy 5 presents in various phenotypes, ranging from a clinically normal fetus to fetuses presenting uterine growth restriction, congenital heart anomalies, multiple dysmorphic features and psychomotor development abnormalities. Although rare, there are cases of a normal psychomotor development regardless of the associated low fetal growth frequently associated with mosaic trisomy 5. This is the first case report to date of a live fetus with complete trisomy 5 reported in chorionic villus sampling and mosaic trisomy 5 in amniotic fluid with a concomitant Ebstein anomaly. Diagnosis of mosaic trisomy 5 represents a challenge for the clinical team and patients, as the information regarding this syndrome is scarce and based mostly on case reports of liveborns, which may introduce a selection bias when counselling the parents.
Topics: Female; Pregnancy; Humans; Chorionic Villi Sampling; Trisomy; Amniotic Fluid; Ebstein Anomaly; Mosaicism; Fetus
PubMed: 38160022
DOI: 10.1136/bcr-2023-256474 -
Journal of Cardiology Cases Dec 2023In this case report, we describe a 23-year-old male with Ebstein's anomaly who experienced out-of-hospital cardiac arrest due to commotio cordis following cliff diving....
UNLABELLED
In this case report, we describe a 23-year-old male with Ebstein's anomaly who experienced out-of-hospital cardiac arrest due to commotio cordis following cliff diving. The patient previously underwent a Cone procedure and re-do reduction tricuspid valvuloplasty. Comprehensive investigations revealed no new ischemic events or structural abnormalities. He received an implantable cardioverter-defibrillator during an uneventful outpatient visit. This is the first reported case of commotio cordis in a patient with Ebstein's anomaly, suggesting a potential increased risk in individuals with congenital heart diseases. This highlights the significance of tertiary prevention in such cases.
LEARNING OBJECTIVE
Through this case, readers may be able to review the incidence and electrical abnormalities leading to sudden cardiac death in patients with commotio cordis, the clinical presentation and mechanism of injury, and the current consensus regarding the management of commotio cordis.
PubMed: 38126049
DOI: 10.1016/j.jccase.2023.08.012