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Journal of Forensic Sciences May 2018A 13-year-old girl is reported who died suddenly and unexpectedly in her sleep from previously undiagnosed Ebstein anomaly. At autopsy, there was dilatation of the right...
A 13-year-old girl is reported who died suddenly and unexpectedly in her sleep from previously undiagnosed Ebstein anomaly. At autopsy, there was dilatation of the right atrium with marked dilatation of the right auricle and apical displacement of the tricuspid valve into the right ventricular cavity with atrialization of the upper portion of the right ventricle. There were also prominent dysplastic changes in both the septal and posterior leaflets of the tricuspid valve with thickening of the valve and fusion of leaflets to the wall of the ventricle. Histology of the myocardium showed focal, minor microscopic areas of interstitial fibrosis with marked fibrous dysplasia and thickening of the tricuspid valve. Lethal arrhythmias occur in this condition because of the geographical relationship of the conduction system to the abnormal anatomical structures. As adolescents who died suddenly are often minimally symptomatic, cases will rarely present de novo to forensic autopsy.
Topics: Adolescent; Death, Sudden; Ebstein Anomaly; Female; Fibrosis; Heart Atria; Humans; Myocardium; Tricuspid Valve
PubMed: 28940541
DOI: 10.1111/1556-4029.13652 -
Cardiology in the Young Jan 2015
Review
Topics: Diagnosis, Differential; Ebstein Anomaly; Echocardiography; Humans; Tricuspid Valve
PubMed: 25165895
DOI: 10.1017/S1047951114001565 -
JACC. Clinical Electrophysiology Oct 2018Ebstein's anomaly of the tricuspid valve is a relatively rare form of congenital heart disease that has long been a challenge to electrophysiologists and cardiac... (Review)
Review
Ebstein's anomaly of the tricuspid valve is a relatively rare form of congenital heart disease that has long been a challenge to electrophysiologists and cardiac surgeons. In addition to the hemodynamic burden of the actual valve defect, Ebstein's patients must also contend with an extraordinarily high incidence of tachyarrhythmias, most of which can be attributed to accessory atrioventricular pathways (APs) located along the posterior and septal border of the tricuspid valve where the valve leaflets are most abnormal. It is the only congenital heart defect with such a dramatic predisposition toward APs. Although it is logical to postulate a link between the anatomic deformity and the conduction abnormality, the exact nature of this link is still not fully understood and remains a fertile area for investigation that might shed light on abnormal conduction pathways in many other forms of heart disease. Furthermore, for reasons that are only now being fully appreciated, successful catheter ablation of APs in this setting is frequently more challenging than would be expected in a structurally normal heart. This review will explore the gross and microscopic anatomy of Ebstein's anomaly with attention to features that could be important to both arrhythmogenesis and ablation therapy in this unique population.
Topics: Adolescent; Adult; Child; Child, Preschool; Ebstein Anomaly; Humans; Tachycardia; Tricuspid Valve; Young Adult
PubMed: 30336873
DOI: 10.1016/j.jacep.2018.05.024 -
Cardiology in the Young Mar 2024CHD refers to structural cardiac abnormalities which comprise the commonest group of congenital malformations. Malta is a small island in the central Mediterranean with...
BACKGROUND
CHD refers to structural cardiac abnormalities which comprise the commonest group of congenital malformations. Malta is a small island in the central Mediterranean with excellent diagnostic and therapeutic facilities. It is unique in the European population as termination of pregnancy is illegal. This study was carried out to ascertain patterns in CHD prevalence in comparison with EUROCAT data (European Surveillance of Congenital Anomalies).
METHODS
Anonymised data were obtained from the EUROCAT website for 1993-2020.
RESULTS
There were a total of 22,833,032 births from all EUROCAT Registries, of which 121,697 were from Malta. The prevalence rate for Malta CHD was 32.38/10,000 births (at the higher end of the range). Malta had a significant excess of commoner, comparatively non-severe CHDs. For most of the severe lesions analysed rates reported were higher than EUROCAT average, however, apart from Ebstein's anomaly, they all fell within the ranges reported from the different registries.
DISCUSSION
Wide variations in reported CHD prevalence are known, and the Malta rates may be higher for milder defects due to quicker pickup prior to spontaneous resolution. There may also be a higher pickup of milder forms of more severe conditions. For the more severe conditions, lack of termination may be the explanation. These factors may result in the higher neonatal mortality observed in Malta.
Topics: Infant, Newborn; Female; Pregnancy; Humans; Malta; Ebstein Anomaly; Heart Diseases; Infant Mortality; Parturition
PubMed: 37559388
DOI: 10.1017/S1047951123003062 -
Interactive Cardiovascular and Thoracic... Apr 2021Our goal was to evaluate the impact of the adult congenital heart disease anatomical and physiological (ACHD AP) classification system on the surgical management of...
OBJECTIVES
Our goal was to evaluate the impact of the adult congenital heart disease anatomical and physiological (ACHD AP) classification system on the surgical management of Ebstein anomaly (EA) in adult patients.
METHODS
From February 2000 through August 2017, data of patients aged at least 16 years, who underwent primary EA surgery, were retrospectively evaluated. The cohort was divided in 2 groups according to their ACHD AP classification: the moderate EA group (IIB, IIC) and the severe EA group (IID). Survival, freedom from reoperation and freedom from occurrence of major adverse advents were estimated.
RESULTS
There were 33 patients (21 women, 12 men). Eighteen belonged to the moderate group, 15 to the severe group. There were 12 female patients (80%) in the severe group. Patients in the moderate group were younger than those in the severe group (P = 0.02): 32 ± 12 vs 44 ± 15 years old. Thirty tricuspid valve repairs and 3 replacements were performed. Repair was mainly performed in the moderate group (P = 0.02). Overall survival was 90.1 ± 5.4% at 9 months after the operation and did not change in the later follow-up period. It was 100% for patients in the moderate group and 80.0 ± 10.3% in the severe group (P = 0.07), and 75.0 ± 12.5% for female patients of in the severe group compared to 100% for the remaining patients (P = 0.025). Survival free from major adverse events, including reoperation, at 10 years was 60.0 ± 12.6% in the moderate and 38.1% ± 12.9% in the severe group (P = 0.03). No patient in the moderate group evolved to be in the severe group at late follow-up.
CONCLUSION
Adult EA patients should undergo surgery earlier when they are still in the moderate ACHD AP classification.
Topics: Cardiac Surgical Procedures; Ebstein Anomaly; Female; Heart Defects, Congenital; Humans; Male; Retrospective Studies; Treatment Outcome; Tricuspid Valve Insufficiency
PubMed: 33313770
DOI: 10.1093/icvts/ivaa294 -
Pediatric Cardiology Jan 2023With the trend towards childhood surgery in patients with Ebstein anomaly (EA), thorough imaging is crucial for patient selection. This study aimed to assess...
With the trend towards childhood surgery in patients with Ebstein anomaly (EA), thorough imaging is crucial for patient selection. This study aimed to assess biventricular function by echocardiography and cardiac magnetic resonance (CMR) and compare EA severity classifications. Twenty-three patients (8-17 years) underwent echocardiography and CMR. Echocardiographic parameters included tricuspid annular plane systolic excursions (TAPSE), fractional area change of the functional right ventricle (fRV-FAC), fRV free wall peak systolic myocardial velocity (fRVs'), and tricuspid regurgitation (TR). End-diastolic and end-systolic volume (EDV resp. ESV), fRV- and LV ejection fraction (EF) and TR were obtained by CMR. EA severity classifications included displacement index, Celermajer index and the total-right/left-volume index. Median fRV-FAC was 38% (IQR 33-42). TAPSE and fRVs' were reduced in 39% and 75% of the patients, respectively. Echocardiographic TR was visually graded as mild, moderate, or severe in nine, six and eight patients, respectively. By CMR, median fRVEF was 49% (IQR 36-58) and TR was graded as mild, moderate, or severe in nine, twelve and two patients, respectively. In 70% of cases, fRV-EDV was higher than LV-EDV. LVEF was decreased in 17 cases (74%). There was excellent correlation between echocardiography-derived fRV-FAC and CMR-derived fRVEF (rho = 0.812, p < 0.001). While echocardiography is a versatile tool in the complex geometry of the Ebstein heart, it has limitations. CMR offers a total overview and has the advantage of reliable volume assessment of both ventricles. Comprehensive evaluation of pediatric patients with EA may therefore require a synergistic implementation of echocardiography and CMR.
Topics: Adolescent; Child; Humans; Ebstein Anomaly; Echocardiography; Heart Ventricles; Magnetic Resonance Imaging; Reproducibility of Results; Tricuspid Valve Insufficiency; Stroke Volume
PubMed: 35727332
DOI: 10.1007/s00246-022-02948-3 -
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 -
Heart (British Cardiac Society) Jan 2018Patients with Ebstein's anomaly of the tricuspid valve (EA) are at risk of tachyarrhythmia, congestive heart failure and sudden cardiac death. We sought to determine the...
OBJECTIVES
Patients with Ebstein's anomaly of the tricuspid valve (EA) are at risk of tachyarrhythmia, congestive heart failure and sudden cardiac death. We sought to determine the value of cardiovascular magnetic resonance (CMR) for predicting these outcomes.
METHODS
Seventy-nine consecutive adult patients (aged 37±15 years) with unrepaired EA underwent CMR and were followed prospectively for a median 3.4 (range 0.4-10.9) years for clinical outcomes, namely major adverse cardiovascular events (MACEs: sustained ventricular tachycardia/heart failure hospital admission/cardiac transplantation/death) and first-onset atrial tachyarrhythmia (AT).
RESULTS
CMR-derived variables associated with MACE (n=6) were right ventricular (RV) or left ventricular (LV) ejection fraction (EF) (HR 2.06, 95% CI 1.168 to 3.623, p=0.012 and HR 2.35, 95% CI 1.348 to 4.082, p=0.003, respectively), LV stroke volume index (HR 2.82, 95% CI 1.212 to 7.092, p=0.028) and cardiac index (HR 1.71, 95% CI 1.002 to 1.366, p=0.037); all remained significant when tested solely for mortality. History of AT (HR 11.16, 95% CI 1.30 to 95.81, p=0.028) and New York Heart Association class >2 (HR 7.66, 95% CI 1.54 to 38.20, p=0.013) were also associated with MACE; AT preceded all but one MACE, suggesting its potential role as an early marker of adverse outcome (p=0.011).CMR variables associated with first-onset AT (n=17; 21.5%) included RVEF (HR 1.55, 95% CI 1.103 to 2.160, p=0.011), total R/L volume index (HR 1.18, 95% CI 1.06 to 1.32, p=0.002), RV/LV end diastolic volume ratio (HR 1.55, 95% CI 1.14 to 2.10, p=0.005) and apical septal leaflet displacement/total LV septal length (HR 1.03, 95% CI 1.00 to 1.07, p=0.041); the latter two combined enhanced risk prediction (HR 6.12, 95% CI 1.67 to 22.56, p=0.007).
CONCLUSION
CMR-derived indices carry prognostic information regarding MACE and first-onset AT among adults with unrepaired EA. CMR may be included in the periodic surveillance of these patients.
Topics: Adult; Ebstein Anomaly; Female; Follow-Up Studies; Forecasting; Heart Ventricles; Humans; Magnetic Resonance Imaging, Cine; Male; Predictive Value of Tests; Prognosis; Prospective Studies; Tachycardia, Supraventricular; Ventricular Function, Left
PubMed: 28684436
DOI: 10.1136/heartjnl-2017-311274 -
The Journal of Thoracic and... Dec 2020
Topics: Ebstein Anomaly; Humans
PubMed: 32763030
DOI: 10.1016/j.jtcvs.2020.06.075