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Indian Journal of Thoracic and... May 2024Ebstein's anomaly is a rare congenital cardiac disease which is often associated with various other cardiac anomalies. However, its association with total anomalous...
UNLABELLED
Ebstein's anomaly is a rare congenital cardiac disease which is often associated with various other cardiac anomalies. However, its association with total anomalous pulmonary venous connection is extremely rare with only one case reported so far in the English literature. We report the first successful surgical correction of both Ebstein's anomaly and total anomalous pulmonary venous connection in an adult patient. Such complex scenarios may pose unique challenges in management which require a judicious approach.
SUPPLEMENTARY INFORMATION
The online version contains supplementary material available at 10.1007/s12055-023-01664-8.
PubMed: 38681710
DOI: 10.1007/s12055-023-01664-8 -
Journal of Clinical Ultrasound : JCU Apr 2024To analyze the influence of RV dysfunction evaluated by Free-angle M-mode (FAM) TAPSE Z-score on retrograde ductus arteriosus flow (RDAF) in fetuses with Ebstein anomaly...
Study on the correlation between retrograde ductus arteriosus flow and right ventricular function evaluated by Z-score of tricuspid annular plane systolic excursion in fetuses with Ebstein anomaly.
PURPOSE
To analyze the influence of RV dysfunction evaluated by Free-angle M-mode (FAM) TAPSE Z-score on retrograde ductus arteriosus flow (RDAF) in fetuses with Ebstein anomaly (EA).
METHODS
A retrospective cohort study of 30 EA and 60 normal fetuses were enrolled. The EA group was divided into two groups: with RDAF (EA-RDAF group) and without RDAF (EA-NRDAF group). FAM was used to measure TAPSE of EA and normal fetuses, and Z-scores were calculated. The differences of FAM-TAPSE Z-score, gestational week (GW), maternal age (MA), and mitral valve-tricuspid valve distance (MTD) between three groups were compared. The correlation and binary logistic regression between FAM-TAPSE Z-score, GW, MA, MTD, and RDAF were analyzed.
RESULTS
FAM-TAPSE Z-score was significantly lower in EA-RDAF group compared to other groups (p < 0.05). FAM-TAPSE Z-score, GW, and MA were negatively correlated with RDAF (p < 0.05), but no correlation was found between TR, MDT, and RDAF (p > 0.05). Multivariate logistic regression showed that FAM-TAPSE Z-score was an independent influencing factor for RDAF (OR = 0.102, p < 0.05).
CONCLUSION
RV dysfunction is an independent factor leading to RDAF in EA fetus, which provides a feasible theoretical basis for further study on improvement of RV function through intrauterine treatment to delay and prevent the RDAF, to avoid death cycle and improve live-birth rate.
PubMed: 38655706
DOI: 10.1002/jcu.23695 -
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 Cardiovascular Medicine... Jul 2024
Topics: Humans; Foramen Ovale, Patent; Ebstein Anomaly; Septal Occluder Device; Cardiac Catheterization; Treatment Failure; Female; Male; Echocardiography, Transesophageal; Adult
PubMed: 38625819
DOI: 10.2459/JCM.0000000000001615 -
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 -
CJC Open Mar 2024
PubMed: 38559330
DOI: 10.1016/j.cjco.2023.12.009