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Heart (British Cardiac Society) Jan 2024Although survival has significantly improved in the last four decades, the diagnosis of Ebstein's anomaly is still associated with a 20-fold increased risk of mortality,... (Review)
Review
Although survival has significantly improved in the last four decades, the diagnosis of Ebstein's anomaly is still associated with a 20-fold increased risk of mortality, which generally drops after neonatal period and increases subtly thereafter. With increasing age of presentation, appropriate timing of intervention is challenged by a wide spectrum of disease and paucity of data on patient-tailored interventional strategies. The present review sought to shed light on the wide grey zone of post-neonatal Ebstein's manifestations, highlighting current gaps and achievements in knowledge for adequate risk assessment and appropriate therapeutic strategy.A 'wait-and-see' approach has been adopted in many circumstances, though its efficacy is now questioned by the awareness that Ebstein's anomaly is not a benign disease, even when asymptomatic. Moreover, older age at intervention showed a negative impact on post-surgical outcome.In order to tackle the extreme heterogeneity of Ebstein's anomaly, this review displays the multimodality imaging assessment necessary for a proper anatomical classification and the multidisciplinary approach needed for a comprehensive risk stratification and monitoring strategy. Currently available predictors of clinical outcome are summarised for both operated and unoperated patients, with the aim of supporting the decisional process on the choice of appropriate therapy and optimal timing for intervention.
Topics: Infant, Newborn; Child; Adult; Humans; Ebstein Anomaly; Risk Assessment; Multimodal Imaging
PubMed: 37487694
DOI: 10.1136/heartjnl-2023-322420 -
Cardiovascular Diagnosis and Therapy Dec 2021Ebstein's anomaly is a rare congenital heart disease with malformation of the tricuspid valve and myopathy of the right ventricle. The septal and inferior leaflets... (Review)
Review
Ebstein's anomaly is a rare congenital heart disease with malformation of the tricuspid valve and myopathy of the right ventricle. The septal and inferior leaflets adhere to the endocardium due to failure of delamination. This leads to apical displacement of their hinge points with a shift of the functional tricuspid valve annulus towards the right ventricular outflow tract with a possibly restrictive orifice. Frequently, a coaptation gap yields tricuspid valve regurgitation and over time the "atrialized" portion of the right ventricle may dilate. The highly variable anatomy determines the clinical presentation ranging from asymptomatic to very severe with need for early operation. Echocardiography and magnetic resonance imaging are the most important diagnostic modalities to assess the tricuspid valve as well as ventricular morphology and function. While medical management of asymptomatic patients can be effective for many years, surgical intervention is indicated before development of significant right ventricular dilatation or dysfunction. Onset of symptoms and arrhythmias are further indications for surgery. Modified cone reconstruction of the tricuspid valve is the state-of-the-art approach yielding the best results for most patients. Alternative procedures for select cases include tricuspid valve replacement and bidirectional cavopulmonary shunt depending on patient age and other individual characteristics. Long-term survival after surgery is favorable but rehospitalization and reoperation remain significant issues. Further studies are warranted to identify the optimal surgical strategy and timing before adverse right ventricular remodeling occurs. It is this article's objective to provide a comprehensive review of current literature and an overview on the management of Ebstein's Anomaly. It focuses on imaging, cardiac surgery, and outcome. Additionally, a brief insight into arrhythmias and their management is given. The "future perspectives" summarize open questions and fields of future research.
PubMed: 35070800
DOI: 10.21037/cdt-20-771 -
JTCVS Techniques Sep 2020
PubMed: 34317902
DOI: 10.1016/j.xjtc.2020.05.024 -
Annals of Cardiothoracic Surgery May 2017Ebstein malformation is a rare disease that presents with a spectrum of severity. We use a management paradigm that breaks the anomaly into two groups: neonates and...
Ebstein malformation is a rare disease that presents with a spectrum of severity. We use a management paradigm that breaks the anomaly into two groups: neonates and children/adults. This leads to a discussion of management and operative techniques as well as pitfalls. We hope the reader finds our streamlined and logical approach of benefit to this complex and rare disease.
PubMed: 28706870
DOI: 10.21037/acs.2017.05.03 -
Internal Medicine (Tokyo, Japan) Dec 2022Ebstein's anomaly is an uncommon congenital disorder affecting the tricuspid valve. We herein report a 38-year-old woman who experienced consciousness and sensory...
Ebstein's anomaly is an uncommon congenital disorder affecting the tricuspid valve. We herein report a 38-year-old woman who experienced consciousness and sensory disturbance during treatment for heart failure caused by Ebstein's anomaly. Urgent magnetic resonance imaging and cerebral angiography demonstrated acute cerebral infarction and internal carotid artery obstruction with the development of collateral arteries. We diagnosed her with multiple cerebral infarctions due to moyamoya disease. Ebstein's anomaly concomitant with moyamoya disease is extremely rare. However, we should consider the possibility of this rare but important concurrence when treating patients with heart failure due to Ebstein's anomaly to avoid excessive diuresis and vasodilation and irreversible brain injury.
Topics: Female; Humans; Adult; Ebstein Anomaly; Moyamoya Disease; Tricuspid Valve; Heart Failure
PubMed: 35569986
DOI: 10.2169/internalmedicine.9516-22 -
The Journal of Thoracic and... Mar 2021To evaluate late-term tricuspid valve competence and biventricular function following cone reconstruction for Ebstein anomaly, and to explore biventricular remodeling.
OBJECTIVES
To evaluate late-term tricuspid valve competence and biventricular function following cone reconstruction for Ebstein anomaly, and to explore biventricular remodeling.
METHODS
Consecutive adult and pediatric patients who underwent cone reconstruction from 2009 to 2019 were reviewed for inclusion in this retrospective cardiac magnetic resonance imaging study. Tricuspid valve competence was assessed with tricuspid regurgitation fraction. Biventricular systolic function was assessed by ejection fraction, cardiac index, indexed stroke volume, and indexed aortic and pulmonary artery beat volume. Biventricular remodeling was assessed by planimetered areas (right atrium, functional right ventricle, left heart), and indexed end-diastolic and end-systolic ventricular volumes. Paired t tests or Wilcoxon signed-rank tests were used for analyses.
RESULTS
Of 58 included patients, 50 underwent cardiac magnetic resonance imaging. Twelve patients had both preoperative and late postoperative cardiac magnetic resonance imaging with a median follow-up of 5.11 years (interquartile range, 3.12-6.07 years). Focusing on these, tricuspid regurgitation fraction decreased (from 69% to 10%; P = .014), right ventricle ejection fraction remained stable, and antegrade pulmonary artery beat volume increased (from 26.7 to 41.6 mL/beat/m; P = .037). The left ventricle stroke volume (from 30.4 to 44.1 mL/m; P = .015) and antegrade aortic beat volume (from 28.5 to 41.1 mL/beat/m; P = .014) also increased, and the left ventricle stroke volume improved progressively with time since surgery (P = .048). Whereas the right atrium area decreased (P = .004), the functional right ventricle and left heart area increased (cm, P = .021 and P = .004). Right ventricle volumes showed a tendency to normalize and left ventricle indexed end-diastolic volume increased (from 50 to 69 mL/m; P = .03) over time.
CONCLUSIONS
Cone valve integrity was sustained. Biventricular function improved progressively during follow-up, and there are positive signs of biventricular remodeling late after cone reconstruction.
Topics: Adolescent; Cardiac Surgical Procedures; Child; Child, Preschool; Databases, Factual; Ebstein Anomaly; Female; Humans; London; Magnetic Resonance Imaging; Male; Recovery of Function; Retrospective Studies; Time Factors; Treatment Outcome; Tricuspid Valve; Ventricular Function, Left; Ventricular Function, Right; Ventricular Remodeling; Young Adult
PubMed: 33293067
DOI: 10.1016/j.jtcvs.2020.10.124 -
The Journal of Thoracic and... Mar 2021
Topics: Ebstein Anomaly; Humans
PubMed: 33431212
DOI: 10.1016/j.jtcvs.2020.12.004 -
Veterinary Research Forum : An... 2022A one and a half years old male French bulldog weighing 9.50 kg was presented with the history of inappetence, lethargy, abdominal distension and exercise intolerance...
A one and a half years old male French bulldog weighing 9.50 kg was presented with the history of inappetence, lethargy, abdominal distension and exercise intolerance since last 2 days. The physical examination was done which revealed normal physiological parameters including temperature, mucus membrane color and capillary refill time except palpable precordial thrills, jugular distension on palpation, tachycardia and systolic murmurs on auscultation. Electrocardiography (ECG) was done which depicted ectopic foci with atrioventricular junctional tachycardia and right ventricular enlargement involving very small inverted P waves, deep S waves in leads I, II, III and augmented vector foot (aVF) and splintered QRS complexes. The dog was undergone chest radiography that revealed right atrial enlargement, increased sternal contact of heart on lateral view and a bulge at 9:00 o'clock to 11:00 o'clock depicted right atrial enlargement on dorso-ventral view. Lastly, echocardiography was done to arrive at a diagnosis confirming the Ebstein's anomaly as a form of tricuspid valve dysplasia including apical displacement of tricuspid valve leaflets, division of right ventricle into atrialized and functional portions, increased displacement index, increased apex-mitral annulus to apex-tricuspid annulus ratio, severe right atrial dilatation and tricuspid regurgitation. The dog was medically treated with diuretics, angiotensin converting enzyme inhibitors and inotropes and the owner was advised to put the dog on low sodium diet for 2 weeks. The dog has resolved clinical signs of right sided heart affection; but, suddenly collapsed at home. The owner denied for the necropsy of dog.
PubMed: 36686865
DOI: 10.30466/vrf.2022.550981.3425 -
The Turkish Journal of Pediatrics 2017Yuan SM. Congenital heart defects in Williams syndrome. Turk J Pediatr 2017; 59: 225-232. Williams syndrome (WS), also known as Williams-Beuren syndrome, is a rare... (Review)
Review
Yuan SM. Congenital heart defects in Williams syndrome. Turk J Pediatr 2017; 59: 225-232. Williams syndrome (WS), also known as Williams-Beuren syndrome, is a rare genetic disorder involving multiple systems including the circulatory system. However, the etiologies of the associated congenital heart defects in WS patients have not been sufficiently elucidated and represent therapeutic challenges. The typical congenital heart defects in WS were supravalvar aortic stenosis, pulmonary stenosis (both valvular and peripheral), aortic coarctation and mitral valvar prolapse. The atypical cardiovascular anomalies include tetralogy of Fallot, atrial septal defects, aortic and mitral valvular insufficiencies, bicuspid aortic valves, ventricular septal defects, total anomalous pulmonary venous return, double chambered right ventricle, Ebstein anomaly and arterial anomalies. Deletion of the elastin gene on chromosome 7q11.23 leads to deficiency or abnormal deposition of elastin during cardiovascular development, thereby leading to widespread cardiovascular abnormalities in WS. In this article, the distribution, treatment and surgical outcomes of typical and atypical cardiac defects in WS are discussed.
Topics: Child; Child, Preschool; Heart Defects, Congenital; Humans; Williams Syndrome
PubMed: 29376566
DOI: 10.24953/turkjped.2017.03.001 -
JACC. Clinical Electrophysiology Sep 2021
Review
Topics: Arrhythmias, Cardiac; Ebstein Anomaly; Humans; Morphogenesis; Tricuspid Valve
PubMed: 34454887
DOI: 10.1016/j.jacep.2021.05.008