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La Tunisie MedicaleEbstein's disease (ED) is a rare and heterogeneous congenital heart disease affecting the tricuspid valve and the right ventricle. Few studies have analyzed the...
INTRODUCTION
Ebstein's disease (ED) is a rare and heterogeneous congenital heart disease affecting the tricuspid valve and the right ventricle. Few studies have analyzed the electrocardiographic features of this disease.
AIM
To describe the electrocardiographic features observed in Ebstein's disease.
METHODS
We conducted a retrospective descriptive study that enrolled 26 patients followed for ED.
RESULTS
The mean age of discovery of the ME was 103.5±99 months [0-31 years]. The diagnosis of ME is most often made between 5 and 10 years. We noted right atrial hypertrophy in 11 patients (42%), right ventricular hypertrophy in half of the patients. Right axial deviation was noted in 11 patients (42%). Eight patients (30%) had wide QRS≥ 120 ms. Seven of these 8 patients (27%) had a fragmented QRS appearance. A right bandle block was noted in 22 patients (84%), it was a complete block in 7 cases (27%). A preexcitation was found in 6 patients (23%). The localization of accessory pathway was right postero-septal in all cases. Rhythmic disorders were noted in 9 patients (34%). It was a junctional tachycardia in 3 patients (11%), atrial flutter in 4 patients (15%) and atrial fibrillation in 2 patients (7%). A second degree atriventricular block was observed in one patient, it was Mobitz I type. Two cases of postoperative rhythm disturbances were recorded: paroxysmal atrial fibrillation and junctional tachycardia related to Wolf Parkinson White (WPW) syndrome.
CONCLUSION
Surface ECG in the ED is often pathological with prevalence of rhythm disturbances related to WPW syndrome.
Topics: Atrial Fibrillation; Ebstein Anomaly; Humans; Retrospective Studies; Tricuspid Valve; Wolff-Parkinson-White Syndrome
PubMed: 35244913
DOI: No ID Found -
The Journal of Thoracic and... Dec 2020
Topics: Ebstein Anomaly; Humans; Retinal Cone Photoreceptor Cells
PubMed: 32682585
DOI: 10.1016/j.jtcvs.2020.06.049 -
The Journal of Thoracic and... Dec 2020
Topics: Ebstein Anomaly; Humans
PubMed: 32763030
DOI: 10.1016/j.jtcvs.2020.06.075 -
The Journal of Thoracic and... Dec 2020We aimed to investigate tricuspid valve function and adverse events after conventional repair and valve replacement for Ebstein's anomaly and compare them with cone...
OBJECTIVES
We aimed to investigate tricuspid valve function and adverse events after conventional repair and valve replacement for Ebstein's anomaly and compare them with cone repair.
METHODS
The medical records of 151 patients (mean age, 25 years; 62% were female) who underwent operation in a single center from 1985 to 2018 were retrospectively analyzed. To determine tricuspid valve regurgitation during follow-up, serial echocardiographic examination was used (n = 2397, tricuspid regurgitation grades were graphed for every patient).
RESULTS
Thirty-nine patients underwent cone repair, 107 patients underwent other repair techniques, and 5 patients underwent valve replacement. The operative mortality was 1.3% (n = 2). Failed valve repair (defined as in-hospital death, conversion to replacement, or in-hospital reoperation) was less frequent after cone repair than after other repair techniques (5%, n = 2 vs 20%, n = 21, P = .039). Mean follow-up was 12.3 years (cone repair: 3.7 years). The 5-year cumulative incidence of moderate or greater recurrent tricuspid regurgitation was lower after cone repair than after other repair techniques (8% vs 32%, P = .03). Among the patients undergoing other repair techniques, the 15-year cumulative incidence of moderate or greater recurrent tricuspid regurgitation, severe tricuspid regurgitation, and reoperation was 58%, 37%, and 31%, respectively. During follow-up, 18 patients died (13 of cardiac and 5 of noncardiac causes). Among patients who died of cardiac causes, 10 of 13 had all 3 characteristics-moderate or greater tricuspid regurgitation, atrial fibrillation, and New York Heart Association classification III and IV-at their last medical evaluation.
CONCLUSIONS
Before cone repair, recurrent tricuspid regurgitation was considerable. Cone repair provided a higher rate of successful repair and a lower incidence of moderate or greater recurrent tricuspid regurgitation at the midterm follow-up.
Topics: Adult; Cardiac Surgical Procedures; Ebstein Anomaly; Echocardiography; Female; Follow-Up Studies; Forecasting; Humans; Male; Reoperation; Retrospective Studies; Treatment Outcome; Tricuspid Valve; Tricuspid Valve Insufficiency; Ventricular Function, Right
PubMed: 32711971
DOI: 10.1016/j.jtcvs.2020.05.032 -
Journal of Cardiac Surgery Jul 2022Ebstein's anomaly,a rare cardiac disease that accounts for <1% of all congenital heart diseases, is a right ventricular myopathy with varying degrees of delamination in...
BACKGROUND
Ebstein's anomaly,a rare cardiac disease that accounts for <1% of all congenital heart diseases, is a right ventricular myopathy with varying degrees of delamination in the endocardium under the tricuspid valve. Cone reconstruction, first described by da Silva in 2004, is a modification of the Carpentier technique. An alternative surgical treatment for Ebstein's anomaly, it is now the preferred surgical corrective technique.
AIMS
This study demonstrates that our modifications of the cone repair procedure to correct Ebstein's anomaly improve valve coaptation and stabilization in the early and midterm.
MATERIALS AND METHODS
Of the 134 patients diagnosed with Ebstein's anomaly in our clinic between January 2012 and October 2020, 10 underwent a cone procedure and its modifications and were thus included in the study. The mean age of these patients was 28.6 ± 18.79 years and ranged from 1 to 61 years. Thirty percent of the patients were male and 70% were female.
RESULTS
The additional procedures performed were an isolated cone procedure (two patients), anomalous pulmonary venous return repair in addition to a cone procedure (one patient), single-vessel coronary artery bypass (one patient), Glenn procedure (two patients), and tricuspid ring annuloplasty (four patients).
CONCLUSION
Based on the results of this study, which assesses the outcomes of patients who underwent cone repairs with surgical modifications, we argue that right ventricular oblique plication, tricuspid valve delamination, surgical valve rotation, and tricuspid ring annuloplasty protect valve coaptation and function in the early and midterm, thereby having beneficial effects on right ventricle remodeling.
Topics: Adolescent; Adult; Cardiac Valve Annuloplasty; Child; Child, Preschool; Ebstein Anomaly; Female; Heart Ventricles; Humans; Infant; Male; Middle Aged; Tricuspid Valve; Tricuspid Valve Insufficiency; Young Adult
PubMed: 35307869
DOI: 10.1111/jocs.16382 -
Cardiovascular Journal of Africa Mar 2023The aim of this study was to review late results of the surgical treatment of Ebstein's anomaly with reconstruction and replacement in adults.
OBJECTIVE
The aim of this study was to review late results of the surgical treatment of Ebstein's anomaly with reconstruction and replacement in adults.
METHODS
Medical records of 28 consecutive patients operated on between 1991 and 2014 were reviewed retrospectively. Surgical repair was performed in 19 (67.9%) patients (Hardy: two, Danielson: three, modified Danielson: six, Carpentier: three, Kay annuloplasty reinforced with ring: two), whereas tricuspid valve replacement was performed in nine patients (32.1%). Primary long-term outcomes consisted of right ventricular function, survival and freedom from re-operation. We evaluated the additional impacts of residual tricuspid insufficiency and type of surgery on survival.
RESULTS
In-hospital mortality rate was 7.1% ( = 2) due to low cardiac output status and sepsis. Patients showed a significant postoperative decrease in tricuspid regurgitation ( < 0.001), right atrial size ( < 0.001) and pulmonary hypertension ( = 0.002). The mean follow-up time was 140 ± 71.4 months, with a median of 126 months (105 - 192). Late mortality occurred in two patients and there was no significant difference in terms of survival based on residual tricuspid insufficiency ( = 0.57) and type of surgery ( = 0.094). Overall survival rates were 89.3, 85.4, 85.4 and 68.3% at five, 10, 15 and 20 years, respectively.
CONCLUSIONS
Although complex leaflet reconstruction techniques have evolved to achieve a more physiological and durable repair, both approaches can be performed safely on specific patients and can be alternated, with acceptable rates of survival and re-operation.
PubMed: 36974890
DOI: 10.5830/CVJA-2023-008 -
Journal of Pediatric Genetics Dec 2021Trisomy 21 is considered the most common chromosomal aneuploidy, and congenital heart disease (CHD) is highly prevalent and relevant to the morbidity and mortality of...
Trisomy 21 is considered the most common chromosomal aneuploidy, and congenital heart disease (CHD) is highly prevalent and relevant to the morbidity and mortality of these patients. Ebstein anomaly (EA) is a rare CHD characterized by tricuspid valve dysplasia with inferior septal leaflet displacement. Herein, we described a patient with trisomy 21 who presented with EA and discuss the association between the two conditions based on a literature review. We conclude that the concomitant occurrence of both conditions is considered to be rare. These individuals are most frequently diagnosed during birth and childhood, and they usually have a good prognosis, as observed with our patient and is typical for EA patients in general. However, it is important to be aware that electrophysiologic anomalies may also be present.
PubMed: 34849279
DOI: 10.1055/s-0040-1714360 -
Seminars in Perinatology Jun 2022Right ventricular outflow tract (RVOT) anomalies comprise a wide spectrum of congenital heart disease, typically characterized by obstruction to flow from the right... (Review)
Review
Right ventricular outflow tract (RVOT) anomalies comprise a wide spectrum of congenital heart disease, typically characterized by obstruction to flow from the right ventricle to pulmonary arteries. This review highlights important considerations surrounding management strategy as well as clinical outcomes for the neonate with RVOT anomaly, including: pulmonary atresia with intact ventricular septum, congenital pulmonary valve stenosis, tetralogy of Fallot, and Ebstein anomaly with anatomic or physiologic RVOT obstruction.
Topics: Heart Defects, Congenital; Heart Ventricles; Humans; Infant, Newborn; Pulmonary Valve; Tetralogy of Fallot; Treatment Outcome; Ventricular Outflow Obstruction
PubMed: 35422353
DOI: 10.1016/j.semperi.2022.151583 -
Open Heart Aug 2023Ebstein's anomaly is a rare congenital cardiac condition and data regarding pregnancy outcomes in this patient group are scarce. We evaluated the maternal and perinatal...
OBJECTIVE
Ebstein's anomaly is a rare congenital cardiac condition and data regarding pregnancy outcomes in this patient group are scarce. We evaluated the maternal and perinatal risks of pregnancy in 81 women with Ebstein's anomaly.
METHODS
The Registry of Pregnancy and Cardiac disease is a prospective global registry of pregnancies in women with structural cardiac disease. Pregnancy outcomes in women with Ebstein's anomaly were examined. The primary outcome was the occurrence of a major adverse cardiac event (MACE) defined as maternal mortality, heart failure, arrhythmia, thromboembolic event or endocarditis. Secondary endpoints were obstetric and perinatal outcomes and the influence of pregnancy on tricuspid valve regurgitation as well as right atrial and ventricular dimensions.
RESULTS
In the 81 women with Ebstein's anomaly (mean age 29.7±6.1 years, 46.9% nulliparous), MACE occurred in 8 (9.9%) pregnancies, mostly heart failure (n=6). There were no maternal deaths. Prepregnancy signs of heart failure were predictive for MACE. Almost half of the women were delivered by caesarean section (45.7%) and preterm delivery occurred in 24.7%. Neonatal mortality was 2.5% and 4.9% of the infants had congenital heart disease. In the subgroup in which prepregnancy and postpregnancy data were available, there was no difference in tricuspid valve regurgitation grade or right atrial and ventricular dimensions before and after pregnancy.
CONCLUSIONS
Most women with Ebstein's anomaly tolerate pregnancy well, but women with prepregnancy signs of heart failure are at higher risk for MACE during pregnancy and should be counselled accordingly.
Topics: Infant, Newborn; Infant; Humans; Female; Pregnancy; Young Adult; Adult; Ebstein Anomaly; Pregnancy Outcome; Tricuspid Valve Insufficiency; Prospective Studies; Cesarean Section; Atrial Fibrillation; Retrospective Studies; Heart Defects, Congenital; Heart Failure; Registries
PubMed: 37550057
DOI: 10.1136/openhrt-2023-002406