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The Journal of Thoracic and... Dec 2020
Topics: Ebstein Anomaly; Echocardiography; Humans
PubMed: 32665108
DOI: 10.1016/j.jtcvs.2020.06.011 -
JTCVS Techniques Jun 2021
PubMed: 34318251
DOI: 10.1016/j.xjtc.2021.02.029 -
Journal of the American Heart... Nov 2020Background In a recent multicenter study of perinatal outcome in fetuses with Ebstein anomaly or tricuspid valve dysplasia, we found that one third of live-born patients...
Background In a recent multicenter study of perinatal outcome in fetuses with Ebstein anomaly or tricuspid valve dysplasia, we found that one third of live-born patients died before hospital discharge. We sought to further describe postnatal management strategies and to define risk factors for neonatal mortality and circulatory outcome at discharge. Methods and Results This 23-center, retrospective study from 2005 to 2011 included 243 fetuses with Ebstein anomaly or tricuspid valve dysplasia. Among live-born patients, clinical and echocardiographic factors were evaluated for association with neonatal mortality and palliated versus biventricular circulation at discharge. Of 176 live-born patients, 7 received comfort care, 11 died <24 hours after birth, and 4 had insufficient data. Among 154 remaining patients, 38 (25%) did not survive to discharge. Nearly half (46%) underwent intervention. Mortality differed by procedure; no deaths occurred in patients who underwent right ventricular exclusion. At discharge, 56% of the cohort had a biventricular circulation (13% following intervention) and 19% were palliated. Lower tricuspid regurgitation jet velocity (odds ratio [OR], 2.3 [1.1-5.0], 95% CI, per m/s; =0.025) and lack of antegrade flow across the pulmonary valve (OR, 4.5 [1.3-14.2]; =0.015) were associated with neonatal mortality by multivariable logistic regression. These variables, along with smaller pulmonary valve dimension, were also associated with a palliated outcome. Conclusions Among neonates with Ebstein anomaly or tricuspid valve dysplasia diagnosed in utero, a variety of management strategies were used across centers, with poor outcomes overall. High-risk patients with low tricuspid regurgitation jet velocity and no antegrade pulmonary blood flow should be considered for right ventricular exclusion to optimize their chance of survival.
Topics: Blood Flow Velocity; Ebstein Anomaly; Echocardiography; Female; Heart Valve Diseases; Hospital Mortality; Humans; Infant, Newborn; Logistic Models; Male; Perinatal Mortality; Prenatal Diagnosis; Retrospective Studies; Risk Factors; Tricuspid Valve
PubMed: 33076749
DOI: 10.1161/JAHA.120.016684 -
Prenatal Diagnosis Feb 2021Ebstein anomaly and tricuspid valve dysplasia (EA/TVD) carry high perinatal mortality. Past studies have focused on cardiac predictors of mortality; we sought to...
OBJECTIVES
Ebstein anomaly and tricuspid valve dysplasia (EA/TVD) carry high perinatal mortality. Past studies have focused on cardiac predictors of mortality; we sought to describe the fetal echo (FE) extracardiac Dopplers in this cohort and determine their association with perinatal mortality.
METHOD
Fetuses with EA/TVD at 23 centers from 2005-2011 were included for retrospective study. Doppler pattern and velocity of the umbilical artery (UA), umbilical vein (UV), ductus venosus (DV), and middle cerebral artery (MCA) were collected. Bivariate and multivariate analyzes were performed. The primary outcome measure was perinatal mortality, defined as fetal demise or neonatal death.
RESULTS
Of 190 cases that met eligibility criteria, alterations were seen in 50% of UA, 16% of UV, 48% of DV, and 8% of MCA Doppler indices on the last FE (median 27.4 weeks). Independent predictors of perinatal mortality included abnormal UA Doppler pattern of absence or reversed end diastolic flow (OR 9.7) and UV velocity z score <1 (OR 2.5), in addition to diagnosis <32 weeks (OR 4.2) and tricuspid valve (TV) annulus z score ≥6 (OR 5.3).
CONCLUSION
Abnormal UA Doppler pattern and decreased UV velocity are independent predictors of perinatal mortality in EA/TVD fetuses and should be used to refine mortality risk and guide perinatal management.
Topics: Cohort Studies; Ebstein Anomaly; Female; Fetus; Gestational Age; Humans; Infant; Infant Mortality; Infant, Newborn; Pregnancy; Pregnancy Outcome; Retrospective Studies; Tricuspid Valve Insufficiency; Ultrasonography, Doppler
PubMed: 33242215
DOI: 10.1002/pd.5873 -
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 -
BMJ Case Reports Jun 2020Ebstein anomaly is a congenital heart defect with a low prevalence and high mortality in the early stages of life. In medical literature, there is no reported...
Ebstein anomaly is a congenital heart defect with a low prevalence and high mortality in the early stages of life. In medical literature, there is no reported association between Ebstein anomaly and cri du chat syndrome. Here, we report the case of a full-term newborn with a low weight for his age and who had a prenatal diagnosis of Ebstein anomaly and a postnatal diagnosis of cri du chat syndrome and 20q duplication detected on array CGH. The patient required medical treatment with inotropic support, high-frequency ventilation and nitric oxide, with an adequate response. Surgical intervention was not needed.
Topics: Airway Management; Cardiotonic Agents; Chromosome Deletion; Chromosome Duplication; Chromosomes, Human, Pair 20; Cri-du-Chat Syndrome; Diagnosis, Differential; Ebstein Anomaly; Genetic Testing; Humans; Infant, Newborn; Male; Neonatal Screening; Nitric Oxide; Patient Care Management; Prenatal Diagnosis; Rare Diseases
PubMed: 32487530
DOI: 10.1136/bcr-2019-233766 -
Revista Medica Del Instituto Mexicano... Jul 2022Ebstein's anomaly is a rare complex congenital heart disease, first described in 1866 by physician Wilhelm Ebstein, characterized by anatomical and functional...
BACKGROUND
Ebstein's anomaly is a rare complex congenital heart disease, first described in 1866 by physician Wilhelm Ebstein, characterized by anatomical and functional malformations of the tricuspid valve and the right ventricle because of inadequate delaminization of the tricuspid valve tissue. By presenting a clinical case and reviewing the literature, we analyzed the approach of an adult patient with Ebstein anomaly with ventricular preexcitation.
CLINICAL CASE
We describe the case of a 34-year-old male patient, with a clinical history of palpitations and dyspnea, and his paraclinics documented Ebstein type B anomaly associated with patent foramen oval and paroxysmal supraventricular tachycardia, Successful radiofrequency ablation was performed and tricuspid valvuloplasty was proposed.
CONCLUSION
It is concluded that Ebstein's anomaly is complex with clinical, morphological and physiopathological heterogeneous spectrum. Manifested from severe symptomatic forms in the neonatal period to asymptomatic or minimally symptomatic ones detected incidentally. Its diagnosis is based on clinical suspicion and confirmed by echocardiography or imaging studies. The therapeutic strategy may be based on medical management in its mild asymptomatic forms up to surgical interventions that include tricuspid valvuloplasty, palliative surgery, valve replacement or cardiac transplantation.
Topics: Adult; Cardiac Surgical Procedures; Ebstein Anomaly; Echocardiography; Humans; Infant, Newborn; Male
PubMed: 35819304
DOI: No ID Found -
BMJ Case Reports Dec 2019We present the case of an infant with prenatal diagnosis, at 32 weeks gestation, of Ebstein's anomaly without anterograde flow from right ventricular to pulmonary...
We present the case of an infant with prenatal diagnosis, at 32 weeks gestation, of Ebstein's anomaly without anterograde flow from right ventricular to pulmonary atresia (PA)-functional PA with flow reversal in the ductus arteriosus. Prostaglandin E1 was started after birth. Chest X-ray showed severe cardiomegaly and echocardiogram confirmed Ebstein's anomaly with a thickened non-opening pulmonary valve without anterograde flow but with mild regurgitation. Multidisciplinary team decision was to progressively reduce prostaglandins and have an expectant attitude. Peripheral oxygen saturation above 85% was maintained and serial echocardiograms documented progressive reduction of the ductus arteriosus and the opening of the pulmonic valve cusps, with the development of anterograde flow. The newborn was discharged at day 19 of life without the need for any intervention, and at last follow-up remains asymptomatic, with anterograde normal flow in the pulmonary valve.
Topics: Aftercare; Alprostadil; Cardiomegaly; Congenital Abnormalities; Ductus Arteriosus; Ebstein Anomaly; Echocardiography; Female; Gestational Age; Heart Defects, Congenital; Humans; Infant, Newborn; Oxygen; Pregnancy; Prenatal Diagnosis; Pulmonary Atresia; Pulmonary Valve; Treatment Outcome; Vasodilator Agents
PubMed: 31888914
DOI: 10.1136/bcr-2019-229809 -
The American Journal of Case Reports Mar 2021BACKGROUND Ebstein's anomaly (EA) is a rare congenital cardiac abnormality with diverse anatomic and spectra of clinical presentations. This heart anomaly occurs in...
BACKGROUND Ebstein's anomaly (EA) is a rare congenital cardiac abnormality with diverse anatomic and spectra of clinical presentations. This heart anomaly occurs in approximately 1 per 200 000 live births and accounts for <1% of congenital cardiac diseases. The main pathologic finding is "atrialization" of the basal region of the right ventricle. EA has been described extensively in the literature; however, to the best of our knowledge, this is the first reported case of EA presenting with "egg-on-stick" appearance. CASE REPORT A 42-year-old woman presented on account of 2 years of progressive abdominal swelling, early satiety, and progressive weight loss. There was an associated history of dyspnea on exertion, easy fatigability, paroxysmal nocturnal dyspnea, and orthopnea. General examination revealed egg-on-stick appearance: visible distended abdominal wall veins and massive ascites with no pedal edema. Overall, pertinent cardiovascular examination findings and echocardiographic features are in keeping with EA. CONCLUSIONS We present the likely first case of egg-on-stick appearance as part of the presentation of EA, which posed a diagnostic dilemma. Echocardiography can help in unraveling this dilemma.
Topics: Adult; Diagnosis, Differential; Ebstein Anomaly; Echocardiography; Female; Heart Defects, Congenital; Heart Ventricles; Humans; Pregnancy
PubMed: 33692328
DOI: 10.12659/AJCR.924810 -
Journal of Clinical Medicine Apr 2024: Ebstein anomaly (EA) is a rare congenital heart disease characterized by the apical displacement of the tricuspid leaflets, creating an enlarged functional right...
A Twenty-Year Follow-Up of Adults with Ebstein Anomaly with Special Focus on Supraventricular Arrhythmias, Supraventricular Arrhythmias and Effectiveness of Catheter Ablation in 20-Year Follow-Up of Adults with Ebstein Anomaly.
: Ebstein anomaly (EA) is a rare congenital heart disease characterized by the apical displacement of the tricuspid leaflets, creating an enlarged functional right atrium. Supraventricular arrhythmias (SVA) are common, and catheter ablation remains challenging. SVA is considered a risk factor for sudden cardiac death in this population. Still, there are very few real-life data on the impact of SVA treated invasively or conservatively on a patient's prognosis. We aimed to analyze the incidence of SVA in adults with EA, evaluate the effectiveness of catheter ablation, and analyze the impact of SVA and catheter ablation on survival in this population. : 71 pts (median age 53 years; range 24-84 years) with EA were evaluated retrospectively from 1988 to 2020. Forty patients (56.3%) had SVA, and eighteen of them (45.0%) required at least one catheter ablation (35 procedures in total). Indications for ablation were mostly intra-atrial reentrant tachycardia (IART) and atrioventricular reentrant tachycardia (AVRT) (14 pts [77.8% and 9 pts [50.0%], respectively. IART and AVRT coexisted in nine pts. One patient suffered from persistent atrial fibrillation. Procedural effectiveness was reported in 28 (80%) cases; over a longer follow-up (mean 12.6 ± 5.4 years), only eight (44.4%) patients were completely free from SVA after the first ablation. In total, 10 patients (14%) died due to cardiovascular events. There was no difference in survival between patients with or without SVA ( = 0.9) and between ablated and non-ablated EA individuals ( = 0.89). : Supraventricular arrhythmia is frequent in adults with Ebstein anomaly. Patients often require more than one catheter ablation but eventually become free from arrhythmias. The imaging parameters assessed by echocardiography or cardiac magnetic resonance do not seem to be associated with ablation outcomes. The impact of supraventricular arrhythmia itself or treatment with radiofrequency ablation is questionable and should be thoroughly investigated in this population.
PubMed: 38610805
DOI: 10.3390/jcm13072039