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The Journal of Thoracic and... Mar 2021
Topics: Ebstein Anomaly; Humans
PubMed: 33431212
DOI: 10.1016/j.jtcvs.2020.12.004 -
Current Treatment Options in... Oct 2014Ebstein anomaly is a developmental abnormality of the tricuspid valve and right ventricle that results in tricuspid regurgitation and right heart enlargement. Because of...
Ebstein anomaly is a developmental abnormality of the tricuspid valve and right ventricle that results in tricuspid regurgitation and right heart enlargement. Because of the variation in clinical severity and associated findings, patients require a detailed, well-tailored evaluation. For these reasons, management of adults with Ebstein anomaly should take place in a center with expertise in adult congenital heart disease. In many patients, the decision regarding if and when to perform surgery remains controversial, largely because of a lack of published data demonstrating improved postoperative symptoms and survival compared to the natural history of the disease. Because standard two-dimensional echocardiography and cardiovascular magnetic resonance imaging planes do not provide the necessary data to preoperatively manage patients, comprehensive echocardiography and cardiovascular magnetic resonance imaging protocols by experts trained in congenital heart disease are essential in the preoperative management of patients with Ebstein anomaly. As patients may be unaware of their exercise limitations, and for prognostic value, serial cardiopulmonary exercise stress testing is very useful in the evaluation of Ebstein anomaly patients. Surgical tricuspid valve repair historically has not been highly successful because of the marked distortion of tricuspid valve leaflets and right ventricular pathology. Over the last several years, reports of newer surgical techniques to repair the valve, with concurrent advances in arrhythmia management of patients hold promise for improved long term outcomes of patients with Ebstein anomaly. However, because Ebstein anomaly is rare and tricuspid valve repair remains technically challenging, the newer valve repair techniques have not yet gained widespread acceptance throughout the adult congenital heart disease community.
PubMed: 25145925
DOI: 10.1007/s11936-014-0338-x -
Circulation. Cardiovascular Imaging Mar 2021
Topics: Ebstein Anomaly; Exercise Tolerance; Fibrosis; Heart Ventricles; Humans; Myocardium
PubMed: 33722058
DOI: 10.1161/CIRCIMAGING.121.012285 -
Journal of Echocardiography Mar 2023
Topics: Humans; Ebstein Anomaly; Heart Ventricles
PubMed: 34436755
DOI: 10.1007/s12574-021-00548-3 -
Brazilian Journal of Cardiovascular... Dec 2019This study aimed to evaluate Ebstein's anomaly surgical correction and its early and long-term outcomes.
OBJECTIVE
This study aimed to evaluate Ebstein's anomaly surgical correction and its early and long-term outcomes.
METHODS
A retrospective analysis of 62 consecutive patients who underwent surgical repair of Ebstein's anomaly in our institution from January 2000 to July 2016. The following long-term outcomes were evaluated: survival, reoperations, tricuspid regurgitation, and postoperative right ventricular dysfunction.
RESULTS
Valve repair was performed in 46 (74.2%) patients - 12 of them using the Da Silva cone reconstruction; tricuspid valve replacement was performed in 11 (17.7%) patients; univentricular palliation in one (1.6%) patient; and the one and a half ventricle repair in four (6.5%) patients. The patients' mean age at the time of surgery was 20.5±14.9 years, and 46.8% of them were male. The mean follow-up time was 8.8±6 years. The 30-day mortality rate was 8.06% and the one and 10-year survival rates were 91.9% both. Eleven (17.7%) of the 62 patients required late reoperation due to tricuspid regurgitation, in an average time of 7.1±4.9 years after the first procedure.
CONCLUSION
In our experience, the long-term results of the surgical treatment of Ebstein's anomaly demonstrate an acceptable survival rate and a low incidence of reinterventions.
Topics: Adolescent; Adult; Aged; Cardiac Surgical Procedures; Child; Child, Preschool; Ebstein Anomaly; Female; Humans; Infant; Kaplan-Meier Estimate; Male; Middle Aged; Postoperative Complications; Reoperation; Retrospective Studies; Severity of Illness Index; Time Factors; Treatment Outcome; Tricuspid Valve; Tricuspid Valve Insufficiency; Ventricular Dysfunction, Right; Young Adult
PubMed: 31364344
DOI: 10.21470/1678-9741-2018-0333 -
International Journal of Cardiology Mar 2019
Topics: Child; Ebstein Anomaly; Echocardiography; Humans; Prognosis
PubMed: 30554929
DOI: 10.1016/j.ijcard.2018.12.015 -
World Journal For Pediatric &... Mar 2024Ebstein anomaly is a rare congenital anomaly of the tricuspid valve which presents challenges to cardiac surgeons due to the spectrum of the disease and the technical...
BACKGROUND
Ebstein anomaly is a rare congenital anomaly of the tricuspid valve which presents challenges to cardiac surgeons due to the spectrum of the disease and the technical difficulty of valve repair. The natural history of the anomaly differs between patients presenting in the neonatal period to those presenting in adulthood.
METHODS
A retrospective review of all patients >15 years of age with Ebstein anomaly, undergoing surgery on the tricuspid valve at 6 centers across Australia and New Zealand was performed. Patients from 1985 to 2019 were included in the study.
RESULTS
A total of 125 patients were included in the study, 76 patients (60%) undergoing tricuspid valve repair, of which 23 patients underwent a Cone repair and 49 (40%) had a tricuspid valve replacement. The mean follow-up was 7.9 ± 7.3 years. Postoperatively, early mortality was 3 patients (2%) and 10-year survival was 91.5%. A postoperative pacemaker was required in 24 patients (19%). Reoperation was required in 21 patients (17%). There was no statistically significant difference in survival or reoperation between patients who underwent repair or replacement of the tricuspid valve; however, with a small number of patients in long-term follow-up.
CONCLUSION
Older children and adult patients undergoing surgery for Ebstein anomaly in Australia and New Zealand experience good medium-term postoperative survival. Repair of the valve is achieved in a significant proportion of patients with increasing use and success with the Cone repair technique.
Topics: Child; Infant, Newborn; Adult; Humans; Adolescent; Ebstein Anomaly; New Zealand; Treatment Outcome; Tricuspid Valve; Australia
PubMed: 38263637
DOI: 10.1177/21501351231189279 -
Congenital Heart Disease Nov 2019Mechanisms and risk factors for cerebrovascular accidents (CVAs) in Ebstein's anomaly (EA) are not well understood; hence, we aimed to clarify these in a large cohort of...
INTRODUCTION
Mechanisms and risk factors for cerebrovascular accidents (CVAs) in Ebstein's anomaly (EA) are not well understood; hence, we aimed to clarify these in a large cohort of EA patients.
METHODS
Patients with a confirmed diagnosis of EA were retrospectively reviewed. Baseline characteristics were compared between patients with and without a prior history of CVA using logistic regression modeling. Cox regression analysis was used to identify predictors of CVA following initial evaluation. CVA incidence from birth and following tricuspid valve surgery were estimated using the Kaplan-Meier method.
RESULTS
Nine hundred sixty-eight patients (median age 21.1 years, 41.5% male) were included, in which, 87 patients (9.0%) had a history of CVA (54 strokes, 33 transient ischemic attacks; 5 associated with brain abscesses) prior to their initial evaluation. The odds of atrial septal defect/patent foramen ovale (odds ratio [OR] 4.91; 95% CI 2.60-21.22; p = .0002) and migraines/headaches (OR 2.38; 95% CI 1.40-4.04; p = .0013) but not atrial arrhythmias (OR 0.75; 95% CI 0.44-1.30; p = .31) were significantly higher among patients with prior CVA following multivariable adjustment. Seventeen patients experienced CVA following initial evaluation; no examined variables including atrial arrhythmias (HR 2.38; 0.91-6.19; p = .076) were predictive of CVA risk. The 10-year, 50-year, and 70-year incidences of CVA were 1.4%, 15.9%, and 23.5%, respectively, with paradoxical embolism heavily implicated.
CONCLUSION
Patients with EA are at substantive risk for CVA. Histories of migraines/headaches and interatrial shunts should prompt concern for paradoxical embolic CVAs. This has significant implications for all patients with atrial-level shunting.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Cardiac Surgical Procedures; Child; Child, Preschool; Ebstein Anomaly; Embolism, Paradoxical; Female; Humans; Incidence; Infant; Infant, Newborn; Male; Middle Aged; Minnesota; Prognosis; Retrospective Studies; Risk Assessment; Risk Factors; Stroke; Time Factors; Young Adult
PubMed: 31545019
DOI: 10.1111/chd.12841 -
Acta Medica Indonesiana Oct 2016A 27-year-old primiparous woman with 28 weeks gestational age was admitted to our hospital with worsening shortness of breath. She was diagnosed with Ebstein's anomaly...
UNLABELLED
A 27-year-old primiparous woman with 28 weeks gestational age was admitted to our hospital with worsening shortness of breath. She was diagnosed with Ebstein's anomaly three years ago, but preferred to be left untreated. The patient was not cyanotic and her vital signs were stable. Her ECG showed incomplete RBBB and prolonged PR-interval. Blood tests revealed mild anemia. Observation of two-dimensional echo with color flow Doppler study showed Ebstein's anomaly with PFO as additional defects, EF of 57%, LV and LA dilatation, RV atrialization, severe TR, and moderate PH with RVSP of 44.3 mmHg. The patient then underwent elective sectio caesaria at 30 weeks of gestational age; both the mother and her baby were alive and were in good conditions.
PROBLEM
there was an increasing breathlessness in this patient so that there was an increasing need to take a decision for her pregnancy.Ebstein's anomaly is a complicated congenital anomaly. Medical treatment may be followed for many years in patients with mild forms of Ebstein's anomaly. Surgery should be considered if there is objective evidence of debasement such as significant enlargement of heart size, reduction of systolic function in echocardiography.
Topics: Adult; Cesarean Section; Diagnosis, Differential; Ebstein Anomaly; Echocardiography, Doppler, Color; Electrocardiography; Female; Humans; Pregnancy; Pregnancy Outcome
PubMed: 28143995
DOI: No ID Found -
Medicina (Kaunas, Lithuania) Nov 2021Ebstein anomaly is a rare congenital heart disease characterized by a varying degree of anatomical and functional abnormalities of tricuspid valve and right ventricle....
Ebstein anomaly is a rare congenital heart disease characterized by a varying degree of anatomical and functional abnormalities of tricuspid valve and right ventricle. It often coexists with other congenital cardiac malformations. Up to 79-89% of patients with Ebstein anomaly have interatrial communication in the form of patent oval foramen or atrial septal defect and more than one-third has other types of cardiac malformations. Association between Ebstein anomaly and right aortic arch is extremely rare and only few cases have been described in the literature so far. Much rarer than with other cardiac malformations, Ebstein anomaly is associated with non-cardiac malformations or genetic syndromes. Several cases of association between Ebstein anomaly and Charge syndrome have been reported, nevertheless, Ebstein anomaly accounts for less than 1% of cardiac defects seen in patients with Charge syndrome. In this case report, we present a unique case of a patient with Charge syndrome where both Ebstein anomaly and right aortic arch are present. The diagnosis of Ebstein anomaly and right aortic arch was established prenatally. In the first years of life, the patient did not exhibit any remarkable symptoms. However, over time, deterioration of right ventricle function and increased tricuspid regurgitation were observed, requiring consideration of surgical treatment at the age of five. In addition, delay in physical, motor, and mental development was observed and thus, at the age of five, the patient was consulted by a medical geneticist and a gene panel to test for structural heart defects was ordered. The test showed a mutation in chromodomain helicase DNA binding protein 7 (CHD7) gene, which, along with clinical features, allowed to establish a diagnosis of Charge syndrome. To the best of the authors' knowledge, this is the first case report of a patient with Charge syndrome, Ebstein anomaly, and right aortic arch that has been described in the literature.
Topics: Aorta, Thoracic; CHARGE Syndrome; DNA Helicases; DNA-Binding Proteins; Ebstein Anomaly; Heart Defects, Congenital; Heart Ventricles; Humans
PubMed: 34833458
DOI: 10.3390/medicina57111239