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Pediatric and Developmental Pathology :... May 2024Ebstein anomaly (EA) is a rare congenital heart defect characterized by abnormal development of the tricuspid valve (TV) and right ventricular myocardium. This study...
Ebstein anomaly (EA) is a rare congenital heart defect characterized by abnormal development of the tricuspid valve (TV) and right ventricular myocardium. This study documents 2 dramatic cases of fetal EA characterized by hydrops and cardiomegaly, leading to intrauterine or early neonatal death. These clinical outcomes were associated with morphological abnormalities including severe tricuspid regurgitation, unguarded TV orifice, pulmonary atresia, and flattened right ventricular myocardium. This study highlights that these adverse anatomical features may result in unfavorable clinical outcomes in fetal EA. While timely identification of such features by prenatal ultrasound is crucial for providing accurate prognostic stratification and guiding treatment decisions, fetopsy may be necessary to discern EA among the spectrum of right-heart anomalies.
PubMed: 38762771
DOI: 10.1177/10935266241250235 -
Pediatric Radiology May 2024High-resolution, isotropic, 3-dimensional (D) data from pediatric cardiovascular computed tomography (CT) offer great potential for the accurate quantitative evaluation... (Review)
Review
High-resolution, isotropic, 3-dimensional (D) data from pediatric cardiovascular computed tomography (CT) offer great potential for the accurate quantitative evaluation of pediatric cardiovascular and pulmonary vascular diseases. Recent pilot studies using pediatric 3-D cardiovascular CT have shown promising results in assessing cardiac function in conditions such as tetralogy of Fallot, cardiac defects with a hypoplastic ventricle, Ebstein anomaly, and in quantifying myocardial mass. In addition, the quantitative assessment of pulmonary vascularity is useful for evaluating differential right-to-left pulmonary vascular volume ratio, the effectiveness of pulmonary angioplasty, and predicting pulmonary hypertension. These initial experiences could broaden the role of pediatric cardiovascular CT in clinical practice. Furthermore, the current barriers to its widespread use, pertinent solutions to these problems, and new applications are discussed. In this review, the 3-D quantitative evaluations of cardiac function and pulmonary vascularity using high-resolution pediatric cardiovascular CT data are illustrated.
PubMed: 38755443
DOI: 10.1007/s00247-024-05931-7 -
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 -
Cardiology in the Young May 2024Ebstein anomaly is frequently associated with accessory pathways, including Mahaim atriofascicular fibres. We herein illustrate successful Mahaim fibre ablation in...
Ebstein anomaly is frequently associated with accessory pathways, including Mahaim atriofascicular fibres. We herein illustrate successful Mahaim fibre ablation in Ebstein anomaly by targeting the ventricular insertion site below the tricuspid ridge.
PubMed: 38712631
DOI: 10.1017/S1047951124025083 -
World Journal For Pediatric &... May 2024Ebstein anomaly is a myopathy of the right ventricle characterized by failure of tricuspid valve delamination with accompanying tricuspid regurgitation. Its rarity,...
Ebstein anomaly is a myopathy of the right ventricle characterized by failure of tricuspid valve delamination with accompanying tricuspid regurgitation. Its rarity, considerable anatomic variability, and frequent absence of symptoms can make the timing of surgery challenging. Contemporary tricuspid repair techniques can be performed with low mortality and bidirectional cavopulmonary anastomosis reduces the risk of operation when right ventricular function is poor. Here, we present a patient who presented late for surgery, failed high-risk conventional surgery and required heart transplantation.
PubMed: 38693812
DOI: 10.1177/21501351241239305 -
Archivos de Cardiologia de Mexico Apr 2024Ebstein anomaly is a congenital defect characterized by a lack of delamination and apical displacement of the tricuspid valve, tricuspid insufficiency, right atrial...
OBJECTIVES
Ebstein anomaly is a congenital defect characterized by a lack of delamination and apical displacement of the tricuspid valve, tricuspid insufficiency, right atrial enlargement, and ventricular dysfunction. To analyze the results and evolution of the different surgical strategies, data were collected from 45 patients operated on during 1990-2018. Twenty-six patients were included with a median age at initial surgery of 11.3 years (range: 13 days-18.6 years).
METHOD
Procedures were plastic 10 patients (38%), cone technique reconstruction 11 (42%), and replacement 5 (19%). Additional interventions were required in 7 patients: cavo-pulmonary anastomosis 4 and Cox-maze 4. Nine patients (34.6%) required reoperation due to severe tricuspid insufficiency. Six had previous plastic, two bioprothesis, and one, cone.
RESULTS
Overall mortality was 11.5% (3)at a median of 10.1 years post-surgery (range: 5.7-10.12) associated with arrhythmias (p = 0.05), right (p = 0.008), left (p = 0.0001) ventricular dysfunction and reoperations (p = 0.03). None were previous conus. Median follow-up was 6.5 years (range: 1-29.1). Ninety-one-point-six percent were in functional class I/II and 79.2% in sinus rhythm.
CONCLUSIONS
The results of the different classic techniques were similar, although not free of complications and reoperations. Cone reconstruction proved to be effective, with low surgical mortality, less need for reoperations, and durability in the medium term.
PubMed: 38687993
DOI: 10.24875/ACM.23000203 -
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 -
The Journal of Thoracic and... Apr 2024Symptomatic neonates and infants with Ebstein anomaly (EA) require complex management. A group of experts was commissioned by the American Association for Thoracic...
OBJECTIVES
Symptomatic neonates and infants with Ebstein anomaly (EA) require complex management. A group of experts was commissioned by the American Association for Thoracic Surgery to provide a framework on this topic focusing on risk stratification and management.
METHODS
The EA Clinical Congenital Practice Standards Committee is a multinational and multidisciplinary group of surgeons and cardiologists with expertise in EA. A citation search in PubMed, Embase, Scopus, and Web of Science was performed using key words related to EA. The search was restricted to the English language and the year 2000 or later and yielded 455 results, of which 71 were related to neonates and infants. Expert consensus statements with class of recommendation and level of evidence were developed using a modified Delphi method, requiring 80% of members votes with at least 75% agreement on each statement.
RESULTS
When evaluating fetuses with EA, those with severe cardiomegaly, retrograde or bidirectional shunt at the ductal level, pulmonary valve atresia, circular shunt, left ventricular dysfunction, or fetal hydrops should be considered high risk for intrauterine demise and postnatal morbidity and mortality. Neonates with EA and severe cardiomegaly, prematurity (<32 weeks), intrauterine growth restriction, pulmonary valve atresia, circular shunt, left ventricular dysfunction, or cardiogenic shock should be considered high risk for morbidity and mortality. Hemodynamically unstable neonates with a circular shunt should have emergent interruption of the circular shunt. Neonates in refractory cardiogenic shock may be palliated with the Starnes procedure. Children may be assessed for later biventricular repair after the Starnes procedure. Neonates without high-risk features of EA may be monitored for spontaneous closure of the patent ductus arteriosus (PDA). Hemodynamically stable neonates with significant pulmonary regurgitation at risk for circular shunt with normal right ventricular systolic pressure should have an attempt at medical closure of the PDA. A medical trial of PDA closure in neonates with functional pulmonary atresia and normal right ventricular systolic pressure (>20-25 mm Hg) should be performed. Neonates who are hemodynamically stable without pulmonary regurgitation but inadequate antegrade pulmonary blood flow may be considered for a PDA stent or systemic to pulmonary artery shunt.
CONCLUSIONS
Risk stratification is essential in neonates and infants with EA. Palliative comfort care may be reasonable in neonates with associated risk factors that may include prematurity, genetic syndromes, other major medical comorbidities, ventricular dysfunction, or sepsis. Neonates who are unstable with a circular shunt should have emergent interruption of the circular shunt. Neonates who are unstable are most commonly palliated with the Starnes procedure. Neonates who are stable should undergo ductal closure. Neonates who are stable with inadequate pulmonary flow may have ductal stenting or a systemic-to-pulmonary artery shunt. Subsequent procedures after Starnes palliation include either single-ventricle palliation or biventricular repair strategies.
PubMed: 38685467
DOI: 10.1016/j.jtcvs.2024.04.018 -
Archives of Gynecology and Obstetrics Apr 2024Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD) represent uncommon congenital malformations of the tricuspid valve. The purpose of this study is to report on...
PURPOSE
Ebstein anomaly (EA) and tricuspid valve dysplasia (TVD) represent uncommon congenital malformations of the tricuspid valve. The purpose of this study is to report on current perinatal outcomes of EA/TVD in our center and to investigate clinical and fetal echocardiographic predictors of perinatal mortality.
METHODS AND RESULTS
We performed a retrospective study among fetuses diagnosed from January 2014 to December 2023. Clinical and echocardiographic data were obtained from hospital records of Research and Education Hospital. The primary outcome was perinatal mortality. Of 21 fetuses diagnosed, there were 1 lost to follow-up, 1 termination, and 7 demises. In the live-born cohort of 12 live-born patients, 2 died before discharge, yielding an overall perinatal mortality of 50%. The median gestational age at diagnosis was 23 for non-survivors and 24 weeks for survivors. Birth weight was lower in non-survivors (2430 g vs 2990 g). Tricuspid insufficiency severity varied insignificantly. Non-survivors exhibited higher rates of hydrops, functional atresia, and absent antegrade flow (p < 0.05). Two infants with severe tricuspid insufficiency and congenital abnormalities died postnatally. The limited dataset enables further analysis for a predictive model. Notably, all non-survivors displayed hydrops, functional atresia, and absent antegrade flow, hindering definitive determination of the most impactful parameter on survival estimation.
CONCLUSION
Perinatal mortality remains notably elevated in fetuses with EA/TVD. The individuals at the highest risk are those with antegrade flow loss and functional atresia of pulmonary valve, this high-risk subgroup could benefit from targeted interventions, such as novel prenatal therapies or a more comprehensive perinatal approach involving optimized timing of delivery and postnatal interventional strategies.
PubMed: 38683395
DOI: 10.1007/s00404-024-07509-y -
Pediatric Critical Care Medicine : a... Apr 2024Multicenter studies reporting outcomes following tracheostomy in children with congenital heart disease are limited, particularly in patients with single ventricle...
Clinical Outcomes After Tracheostomy in Children With Single Ventricle Physiology: Collaborative Research From the Pediatric Cardiac Intensive Care Society Multicenter Cohort, 2010-2021.
OBJECTIVES
Multicenter studies reporting outcomes following tracheostomy in children with congenital heart disease are limited, particularly in patients with single ventricle physiology. We aimed to describe clinical characteristics and outcomes in a multicenter cohort of patients with single ventricle physiology who underwent tracheostomy before Fontan operation.
DESIGN
Multicenter retrospective cohort study.SETTING: Twenty-one tertiary care pediatric institutions participating in the Collaborative Research from the Pediatric Cardiac Intensive Care Society.
PATIENTS
We reviewed 99 children with single ventricle physiology who underwent tracheostomy before the Fontan operation at 21 institutions participating in Collaborative Research from the Pediatric Cardiac Intensive Care Society between January 2010 and December 2020, with follow-up through December 31, 2021.
INTERVENTIONS
None.
MEASUREMENTS AND MAIN RESULTS
Death occurred in 51 of 99 patients (52%). Cox proportional hazard analysis was performed to determine factors associated with death after tracheostomy. Results are presented as hazard ratio (HR) with 95% CIs. Nonrespiratory indication(s) for tracheostomy (HR, 2.21; 95% CI, 1.14-4.32) and number of weeks receiving mechanical ventilation before tracheostomy (HR, 1.06; 95% CI, 1.02-1.11) were independently associated with greater hazard of death. In contrast, diagnosis of tricuspid atresia or Ebstein's anomaly was associated with less hazard of death (HR, 0.16; 95% CI, 0.04-0.69). Favorable outcome, defined as survival to Fontan operation or decannulation while awaiting Fontan operation with viable cardiopulmonary physiology, occurred in 29 of 99 patients (29%). Median duration of mechanical ventilation before tracheostomy was shorter in patients who survived to favorable outcome (6.1 vs. 12.1 wk; p < 0.001), and only one of 16 patients with neurologic indications for tracheostomy and 0 of ten patients with cardiac indications for tracheostomy survived to favorable outcome.
CONCLUSIONS
For children with single ventricle physiology who undergo tracheostomy, mortality risk is high and should be carefully considered when discussing tracheostomy as an option for these children. Favorable outcomes are possible, although thoughtful attention to patient selection and tracheostomy timing are likely necessary to achieve this goal.
PubMed: 38683049
DOI: 10.1097/PCC.0000000000003523