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Clinical Case Reports Jun 2024This case report presents an uncommon variant of Ebstein anomaly, where all three tricuspid valve leaflets exhibited apical displacement-a rare finding. It illustrates...
KEY CLINICAL MESSAGE
This case report presents an uncommon variant of Ebstein anomaly, where all three tricuspid valve leaflets exhibited apical displacement-a rare finding. It illustrates the complexities in diagnosing and managing such atypical presentations, with successful surgical correction through annuloplasty. The report adds valuable insights to the limited literature on this congenital heart disease.
ABSTRACT
Ebstein anomaly (EA), a rare congenital heart disorder, presents with diverse clinical spectrums. This case report explores a distinctive manifestation of EA, where all three tricuspid valve (TV) leaflets exhibited apical displacement, highlighting a novel aspect in the presentation of this condition. A 44-year-old woman, under long-term medical surveillance for EA, showcased an atypical clinical trajectory marked by the apical displacement of all TV leaflets, which is uncommon in EA. Despite a predominantly asymptomatic course, recent exacerbation of symptoms prompted further evaluation. Diagnostic modalities, including echocardiography and cardiac magnetic resonance imaging, revealed severe tricuspid regurgitation concomitant with unprecedented apical displacement of the anterior, septal, and posterior tricuspid leaflets. The displacement of the anterior leaflet was contrary to typical embryonic valvular formation expectations, indicating a unique presentation within EA. The patient underwent annuloplasty surgery, which successfully rectified the anomalous TV architecture. Postoperative evaluation demonstrated mild residual tricuspid regurgitation, and the patient was discharged in stable condition. This case underscores the variability in EA presentations and accentuates the significance of tailored surgical interventions. The observation of apical displacement involving all TV leaflets adds a unique dimension to the existing EA literature, reinforcing the need for careful diagnosis and personalized treatment approaches.
PubMed: 38799521
DOI: 10.1002/ccr3.8991 -
Journal of Cardiovascular Development... May 2024Collaborative multicenter research has significantly increased our understanding of fetal Ebstein anomaly, delineating risk factors for adverse outcomes as well as...
Collaborative multicenter research has significantly increased our understanding of fetal Ebstein anomaly, delineating risk factors for adverse outcomes as well as predictors of postnatal management. These data are incorporated into prenatal care and therapeutic strategies and inform family counseling and delivery planning to optimize care. This report details the translation of findings from multicenter studies into multidisciplinary prenatal care for a fetus with Ebstein anomaly, supraventricular tachycardia, and a circular shunt, including transplacental therapy to control arrhythmias and achieve ductal constriction, informed and coordinated delivery room management, and planned univentricular surgical palliation.
PubMed: 38786969
DOI: 10.3390/jcdd11050147 -
The Journal of Innovations in Cardiac... Apr 2024Ablation of accessory pathways in patients with Ebstein's anomaly can be challenging. Despite increasing experience and advances in mapping technology, success is...
Ablation of accessory pathways in patients with Ebstein's anomaly can be challenging. Despite increasing experience and advances in mapping technology, success is limited and recurrence rates can be high. To date, high-density electroanatomic mapping has not been studied in this anatomical substrate. We present a pediatric case of Ebstein's anomaly in which high-density mapping in Ebstein's anomaly was a useful additional tool to improve the outcome of catheter ablation.
PubMed: 38715551
DOI: 10.19102/icrm.2024.15041 -
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 -
Paradoxical septic embolism in an Ebstein's anomaly patient leading to brain abscess: A case report.Journal of Cardiology Cases Apr 2024Ebstein's anomaly (EA), a congenital cardiac anomaly, is characterized by apical displacement of the tricuspid valve leaflet(s) into the right ventricle. We present the...
UNLABELLED
Ebstein's anomaly (EA), a congenital cardiac anomaly, is characterized by apical displacement of the tricuspid valve leaflet(s) into the right ventricle. We present the case of a 61-year-old female with a history of EA, Wolff-Parkinson-White syndrome, and patent foramen ovale (PFO), who presented with worsening hypoxia and confusion, in the setting of left lower extremity cellulitis and abscess. The computed tomography (CT) scan of the head showed a cerebellar infarct with hemorrhagic conversion. Magnetic resonance imaging of the head showed a satellite lesion raising concern for the embolic nature of infarcts. After ruling out cardioembolic causes of cerebellar infarction, her presenting symptoms were attributed to paradoxical septic emboli from the left leg abscess (demonstrated on CT scan of the leg). She was deemed a poor candidate for surgical closure of PFO due to contraindication to use heparin (due to the presence of hemorrhagic stroke) and underlying comorbidities. Septic embolization is a rare but dreaded complication in EA patients with PFO.
LEARNING OBJECTIVE
•Paradoxical emboli can occur in patients with Ebstein's anomaly (EA) and patent foramen ovale (PFO).•The mainstay of management in case of paradoxical embolism lies with the identification and treatment of the underlying cause, such as infective endocarditis, deep vein thrombosis, or infectious source, as in the present case.•The surgical correction of PFO in EA patients should be considered when the patient becomes symptomatic with cyanosis, hypoxia, or manifestations of paradoxical emboli.
PubMed: 38646075
DOI: 10.1016/j.jccase.2023.12.001 -
JACC. Case Reports Apr 2024Patients with Ebstein anomaly are known to have a higher incidence of interatrial communications and shunting of blood and its components through, mainly due to either...
Patients with Ebstein anomaly are known to have a higher incidence of interatrial communications and shunting of blood and its components through, mainly due to either streaming of tricuspid regurgitation or due to elevated right atrial pressure. Here we describe a case where permanent pacemaker lead kept a patent foramen ovale open leading to right-to-left shunting of blood and exertional hypoxemia. This is the first such case report in the published literature.
PubMed: 38645292
DOI: 10.1016/j.jaccas.2024.102283 -
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 -
Heliyon Apr 2024Multiple accessory pathways (APs) can develop in patients with Ebstein anomaly. Rarely, these APs can participate in antidromic atrioventricular reentrant tachycardia...
Multiple accessory pathways (APs) can develop in patients with Ebstein anomaly. Rarely, these APs can participate in antidromic atrioventricular reentrant tachycardia (AVRT) which can be life-threatening and requires unique considerations for acute management and ultimate ablation. These considerations are discussed herein.
PubMed: 38596017
DOI: 10.1016/j.heliyon.2024.e28895 -
BMC Cardiovascular Disorders Apr 2024Double chambered right ventricle is a rare congenital heart disease that is characterised by the presence of an anomalous muscle bundle that divides the right ventricle...
BACKGROUND
Double chambered right ventricle is a rare congenital heart disease that is characterised by the presence of an anomalous muscle bundle that divides the right ventricle into a low pressure superior (distal) chamber and a high pressure inferior (proximal) chamber. It is found in association with a ventricular septal defect in 90% cases with other associations being tetralogy of Fallot, transposition of great vessels, atrial septal defect and Ebstein's anomaly. On the other hand, subaortic membrane is a form of discrete subaortic stenosis that is characterised by a membranous diaphragm in the subvalvular location of the left ventricular outflow tract. Both of these entities are responsible for causing subvalvular outflow tract obstruction. The occurrence of double chambered right ventricle in association with subaortic membrane is an extremely rare entity with only a few case reports available in the literature.
CASE REPORT
A 13-year-old male child with history of chest pain and palpitations presented to the outpatient department of a tertiary care center. Transthoracic echocardiography revealed a subaortic membrane producing a pressure gradient across the left ventricular outflow tract with dilatation of the right atrium and right ventricle which could not be fully evaluated on echocardiography. Cardiac computed tomography was then performed which additionally revealed an anomalous muscle bundle coursing across the right ventricle from the septum to the subinfundibular region creating a double chambered right ventricle. The patient was then taken up for reconstruction of right ventricular outflow tract and resection of subaortic membrane.
CONCLUSION
Right and left outflow tract obstructions are rare congenital lesions which when seen in combination, become even more infrequent. Echocardiography is a robust tool that detects turbulent flow to identify such lesions. However, poor acoustic window may sometimes result in missing these lesions and computed tomography in such situations can play an important role in detection as well as complete preoperative imaging evaluation.
Topics: Adolescent; Humans; Male; Echocardiography; Heart Defects, Congenital; Heart Septal Defects, Atrial; Heart Septal Defects, Ventricular; Heart Ventricles
PubMed: 38561658
DOI: 10.1186/s12872-024-03842-x -
Heart Rhythm O2 Mar 2024
PubMed: 38560370
DOI: 10.1016/j.hroo.2023.11.005