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Hellenic Journal of Cardiology : HJC =... Jun 2024We aimed to examine biventricular remodeling and function after Ebstein anomaly (EbA) surgical correction using echocardiographic techniques, particularly, the relations...
OBJECTIVE
We aimed to examine biventricular remodeling and function after Ebstein anomaly (EbA) surgical correction using echocardiographic techniques, particularly, the relations between the biventricular changes and the EbA types.
METHODS
From April 2015 to August 2022, 110 patients with EbA were included in this retrospective study based on the Carpentier classification. Echocardiography assessments during the preoperative, early, and mid-term postoperative periods were performed.
RESULTS
The 54 patients with types A and B EbA were included in group 1, whereas the 56 patients with types C and D were in group 2. Seventy-eight patients underwent surgical correction of EbA. The median age at operation was 8.8 years. During the mid-term follow-up, only 9.1% of the patients had moderate or severe tricuspid regurgitation. Right ventricular (RV) systolic function worsened in group 2 at discharge (fractional area change: 27.6 ± 11.2 vs. 35.4 ± 11.5 [baseline], P < 0.05; global longitudinal strain: -10.8 ± 4.4 vs. -17.9 ± 4.7 [baseline], P = 0.0001). RV function slowly recovered at a mean of 12 months of follow-up. Regarding left ventricular (LV) and RV systolic function, no statistical difference was found between before and after surgery in group 1.
CONCLUSION
A high success rate of surgical correction of EbA, with an encouraging durability of the valve, was noted. Biventricular systolic function was maintained fairly in most patients with types A and B postoperatively. A late increase in RV systolic function after an initial reduction and unchanged LV systolic function were observed in the patients with types C and D postoperatively.
PubMed: 38844023
DOI: 10.1016/j.hjc.2024.05.019 -
Journal of the American Society of... Feb 2024Speckle-tracking echocardiography (STE) is now routinely included in cardiac evaluations, but its role in predicting mortality and morbidity in congenital heart disease... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Speckle-tracking echocardiography (STE) is now routinely included in cardiac evaluations, but its role in predicting mortality and morbidity in congenital heart disease (CHD) is not well described. We conducted a systematic review to evaluate the prognostic value of STE in patients with CHD.
METHODS
The EMBASE, Medline, Web of Science, Scopus, and Cochrane Central Register of Controlled Trials (CENTRAL) databases were searched from inception to January 2023 for terms related to all CHD, STE, and prognosis. Meta-analysis of association of right ventricle and left ventricle strain (RV S and LV S, respectively) with major adverse cardiovascular events (MACEs) was performed in atrial switch transposition of the great arteries (asTGA)/congenitally corrected TGA (ccTGA), tetralogy of Fallot (ToF), and congenital aortic stenosis (cAS)/bicuspid aortic valve (BAV). P-value combination analysis was additionally performed for all CHD groups.
RESULTS
A total of 33 studies (30 cohorts, n = 8,619 patients, children, and adults) were included. Meta-analysis showed the following parameters as being associated with MACE: RV S in asTGA/ccTGA (hazard ratio [HR] = 1.1/%; CI, [1.03; 1.18]), RV S and LV S in ToF (HR = 1.14/%; CI, [1.03; 1.26] and HR = 1.14/%; CI, [1.08; 1.2], respectively), and LV S in cAS/BAV (HR = 1.19/%; CI, [1.15; 1.23]). The RV S and strain rate were associated with outcomes also in single ventricle/hypoplastic left heart syndrome (at all palliation stages except before Norwood stage 1) and LV S in Ebstein's anomaly.
CONCLUSIONS
This systematic review and meta-analysis showed that biventricular strain and strain rate were associated with outcomes in a variety of CHD, highlighting the need for updated recommendations on the use of STE in the current guidelines, specific to disease types.
Topics: Adult; Child; Humans; Transposition of Great Vessels; Heart Defects, Congenital; Echocardiography; Tetralogy of Fallot; Heart Ventricles; Heart Atria; Morbidity
PubMed: 37972793
DOI: 10.1016/j.echo.2023.11.003 -
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 -
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 -
Heart (British Cardiac Society) Nov 2023Bioprosthetic valves are often used for pulmonary valve replacement (PVR) and tricuspid valve replacement (TVR) because of concerns about mechanical valve thrombosis in...
BACKGROUND
Bioprosthetic valves are often used for pulmonary valve replacement (PVR) and tricuspid valve replacement (TVR) because of concerns about mechanical valve thrombosis in the right heart. The purpose of this study was to assess prosthetic valve function and outcomes (prosthetic valve dysfunction, reoperations and major bleeding events) after mechanical PVR and TVR and to compare these to bioprostheses implanted in the same positions.
METHOD
Case-control study of adults with congenital heart disease that underwent mechanical TVR or PVR (2003-2021) at Mayo Clinic Rochester, Minnesota. For each mechanical prosthesis, we identified two patients that received bioprosthesis in the same position (1:2 matching).
RESULTS
We identified 48 consecutive patients that underwent mechanical PVR (n=39, age 32 (26-38) years, men 22 (56%)) and/or mechanical TVR (n=17, age 36 (31-42) years, men 9 (53%)), as control group of 78 patients (age 30 (24-36) years, men 44 (56%)) and 34 patients (age 34 (29-39) years, men 18 (53%)) that underwent bioprosthetic PVR and TVR, respectively. The most common diagnoses in patients that received mechanical prosthesis were: tetralogy of Fallot (n=14, 19%), aortic stenosis status post Ross operation (n=11, 23%), truncus arteriosus (n=5, 11%), atrioventricular canal defect (n=4, 8%), Ebstein anomaly (n=3, 6%), double outlet right ventricle (n=2, 4%), valvular pulmonic stenosis (n=2, 4%). Compared with the bioprosthesis group, the mechanical prosthesis group had lower temporal increase in Doppler systolic mean gradient after PVR (∆ -1±2 vs 3±2 mm Hg, p<0.001) and Doppler diastolic mean gradient after TVR (∆ 0±1 vs 2±1 mm Hg, p=0.005). The mechanical prosthesis group also had lower risk of prosthetic valve dysfunction after PVR (1.0% vs 2.8% /year, p=0.02) and after TVR (2.6% vs 4.3% /year, p=0.008), but higher risk of major bleeding events (2.2% vs 0.1% /year, p<0.001).
CONCLUSIONS
Patients that received right-sided mechanical valve prostheses had lower temporal increase in valve gradient, lower risk of prosthetic valve dysfunction, but higher risk of bleeding complications compared with those that underwent right-sided bioprosthetic valve implantation.
Topics: Adult; Male; Humans; Heart Valve Prosthesis Implantation; Case-Control Studies; Heart Defects, Congenital; Heart Valve Prosthesis; Bioprosthesis; Retrospective Studies; Hemorrhage; Treatment Outcome
PubMed: 37407219
DOI: 10.1136/heartjnl-2023-322666 -
HGG Advances Jan 2024Ebstein's anomaly, a rare congenital heart disease, is distinguished by the failure of embryological delamination of the tricuspid valve leaflets from the underlying...
Ebstein's anomaly, a rare congenital heart disease, is distinguished by the failure of embryological delamination of the tricuspid valve leaflets from the underlying primitive right ventricle myocardium. Gaining insight into the genetic basis of Ebstein's anomaly allows a more precise definition of its pathogenesis. In this study, two distinct cohorts from the Chinese Han population were included: a case-control cohort consisting of 82 unrelated cases and 125 controls without cardiac phenotypes and a trio cohort comprising 36 parent-offspring trios. Whole-exome sequencing data from all 315 participants were utilized to identify qualifying variants, encompassing rare (minor allele frequency < 0.1% from East Asians in the gnomAD database) functional variants and high-confidence (HC) loss-of-function (LoF) variants. Various statistical models, including burden tests and variance-component models, were employed to identify rare variants, genes, and biological pathways associated with Ebstein's anomaly. Significant associations were noted between Ebstein's anomaly and rare HC LoF variants found in genes related to the matrisome, a collection of extracellular matrix (ECM) components. Specifically, 47 genes with HC LoF variants were exclusively or predominantly identified in cases, while nine genes showed such variants in the probands. Over half of unrelated cases (n = 42) and approximately one-third of probands (n = 12) were found to carry one or two LoF variants in these prioritized genes. These results highlight the role of the matrisome in the pathogenesis of Ebstein's anomaly, contributing to a better understanding of the genetic architecture underlying this condition. Our findings hold the potential to impact the genetic diagnosis and treatment approaches for Ebstein's anomaly.
Topics: Humans; Ebstein Anomaly; Tricuspid Valve; Heart Defects, Congenital; Myocardium; Heart Ventricles
PubMed: 38006208
DOI: 10.1016/j.xhgg.2023.100258 -
JTCVS Open Dec 2023
PubMed: 38204705
DOI: 10.1016/j.xjon.2023.09.002 -
Journal of Cardiovascular... Dec 2023Acquired Wolff-Parkinson-White (WPW) syndrome can occur after congenital heart disease (CHD) surgery.
INTRODUCTION
Acquired Wolff-Parkinson-White (WPW) syndrome can occur after congenital heart disease (CHD) surgery.
METHODS AND RESULTS
A 27-year-old male with Ebstein's anomaly and manifest WPW syndrome received catheter ablation twice. The first electrophysiology study (EPS) induced orthodromic atrioventricular reentrant tachycardia and successfully eliminated the posteroseptal accessory pathway (AP). Six months after the Cone procedure, the patient suffered from palpitation. The second EPS was performed and abolished the right lateral AP.
CONCLUSION
The appearance of a new AP after the reconstruction of CHD is a rare finding and should raise suspicion of an acquired AP connection.
Topics: Male; Humans; Adult; Accessory Atrioventricular Bundle; Wolff-Parkinson-White Syndrome; Tachycardia, Supraventricular; Arrhythmias, Cardiac; Heart Defects, Congenital; Ebstein Anomaly; Catheter Ablation; Electrocardiography
PubMed: 37964504
DOI: 10.1111/jce.16130 -
Journal of the American Society of... Feb 2024
Topics: Infant; Humans; Tricuspid Valve; Ebstein Anomaly; Heart Ventricles; Critical Illness; Tricuspid Valve Insufficiency
PubMed: 38029862
DOI: 10.1016/j.echo.2023.11.021 -
Cardiology in ReviewThe advancement of medical treatment and surgical technique, along with the invention of cardiopulmonary bypass, has allowed for long-term survival of patients with... (Review)
Review
The advancement of medical treatment and surgical technique, along with the invention of cardiopulmonary bypass, has allowed for long-term survival of patients with cyanotic congenital heart disease (CHD)-with many women with CHD now reaching child-bearing age and wishing to become pregnant. Pregnancy in these women is a major concern as the physiologic adaptations of pregnancy, including an increased circulating volume, increased cardiac output, reduced systemic vascular resistance, and decreased blood pressure, place a substantial load on the cardiovascular system. These changes are essential to meet the increased maternal and fetal metabolic demands and allow for sufficient placental circulation during gestation. However, in women with underlying structural heart conditions, they place an additional hemodynamic burden on the maternal body. Overall, with appropriate risk stratification, pre-conception counseling, and management by specialized cardiologists and high-risk obstetricians, most women with surgically corrected CHDs are expected to carry healthy pregnancies to term with optimization of both maternal and fetal risks. In this article, we describe the current understanding of 5 cyanotic CHDs-Tetralogy of Fallot, Transposition of the Great Arteries, Truncus Arteriosus, Ebstein's Anomaly, and Eisenmenger Syndrome-and explore the specific hemodynamic consequences, maternal and fetal risks, current guidelines, and outcomes of pregnancy in women with these conditions.
Topics: Humans; Pregnancy; Female; Heart Defects, Congenital; Pregnancy Complications, Cardiovascular; Cyanosis
PubMed: 36716356
DOI: 10.1097/CRD.0000000000000512