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JTCVS Techniques Sep 2020
PubMed: 34317896
DOI: 10.1016/j.xjtc.2020.05.033 -
The Korean Journal of Thoracic and... Oct 2020Ebstein anomaly is a rare congenital heart malformation typically involving the tricuspid valve and the right ventricle that has a wide range of anatomical and...
Ebstein anomaly is a rare congenital heart malformation typically involving the tricuspid valve and the right ventricle that has a wide range of anatomical and pathophysiological presentations. Various surgical repair techniques for Ebstein anomaly have been reported because of its near-infinite anatomical variability. Cone repair for Ebstein anomaly can achieve nearly anatomical reconstruction of the tricuspid valve with promising outcomes. In this article, the surgical techniques for cone repair in adult patients with Ebstein anomaly are described in detail, and clinical experiences and technically challenging cases are presented.
PubMed: 33020344
DOI: 10.5090/kjtcs.20.113 -
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 -
Korean Circulation Journal Feb 2024
PubMed: 38346696
DOI: 10.4070/kcj.2024.0007 -
Heart Rhythm O2 Mar 2024
PubMed: 38560370
DOI: 10.1016/j.hroo.2023.11.005 -
Journal of the American Heart... Jan 2024The increasing prevalence of atrial fibrillation (AF) in adults with congenital heart disease raises significant questions regarding its management. The unique... (Review)
Review
The increasing prevalence of atrial fibrillation (AF) in adults with congenital heart disease raises significant questions regarding its management. The unique underlying anatomic and physiological background further adds to the difficulty in eliminating the AF burden in these patients. Herein, we provide an overview of the current knowledge on the pathophysiology and risk factors for AF in adult congenital heart disease, with a special focus on the existing challenges in AF ablation. Emerging imaging modalities and ablation techniques might have a role to play. Evidence regarding the safety and efficacy of AF ablation in adult congenital heart disease is summarized, especially for patients with an atrial septal defect, Ebstein anomaly of the tricuspid valve, tetralogy of Fallot, and Fontan circulation. Finally, any remaining gaps in knowledge and potential areas of future research are highlighted.
Topics: Humans; Adult; Heart Defects, Congenital; Atrial Fibrillation; Heart Septal Defects, Atrial; Catheter Ablation; Ebstein Anomaly
PubMed: 38193287
DOI: 10.1161/JAHA.123.032102 -
Indian Journal of Nuclear Medicine :... 2022Ebstein's anomaly is a rare congenital heart disease. We present the case of a 47-year-old woman with history of Ebstein's anomaly who was referred to nuclear medicine...
Ebstein's anomaly is a rare congenital heart disease. We present the case of a 47-year-old woman with history of Ebstein's anomaly who was referred to nuclear medicine department for a myocardial perfusion single-photon emission computed tomography (SPECT) imaging during pre-anesthetic check-up. Myocardial SPECT perfusion images with (99mTc)-Sestamibi demonstrated medium sizes fixed perfusion defect in the mid and proximal infero-septal wall adjacent inferior wall. This is a cause of potential misinterpretation on myocardial SPECT perfusion imaging as this perfusion defect was due to fibrosis rather than scarred myocardium.
PubMed: 36817212
DOI: 10.4103/ijnm.ijnm_43_22 -
JTCVS Techniques Oct 2021The management of severely symptomatic neonates with Ebstein anomaly is challenging during the early neonatal period. Initial management goals should focus on mitigating...
The management of severely symptomatic neonates with Ebstein anomaly is challenging during the early neonatal period. Initial management goals should focus on mitigating a central shunt; providing respiratory mechanical support; providing an adequate but not excessive source of pulmonary blood flow; and minimizing pulmonary vascular resistance. For most patients thus stabilized, definitive repair should be prudently deferred until it is safe for a bailout bidirectional Glenn anastomosis to be added, usually at age 3 to 4 months. For those who remain critical, initial ligation of the large ductus and placing a more peripheral aortopulmonary shunt, or ligating the main pulmonary artery, should be weighed against a primary biventricular repair (Knott-Craig repair), or the Starnes' single-ventricle palliation. The Da Silva cone biventricular repair should generally be avoided during the early neonatal period. An initial Starnes' repair can be potentially converted to a biventricular repair in later infancy.
PubMed: 34647081
DOI: 10.1016/j.xjtc.2021.05.030 -
JTCVS Open Jun 2023Ebstein's anomaly is a rare congenital heart malformation for which surgical and medical management are still controversial. The cone repair has transformed surgical...
OBJECTIVE
Ebstein's anomaly is a rare congenital heart malformation for which surgical and medical management are still controversial. The cone repair has transformed surgical outcomes in many of these patients. We aimed to present our results on the outcomes of patients with Ebstein's anomaly who underwent a cone repair or tricuspid valve replacement.
METHODS
A total of 85 patients who underwent a cone repair (mean age, 16.5 years) or tricuspid valve replacement (mean age, 40.8 years) between 2006 and 2021 were included. Univariate, multivariate, and Kaplan-Meier analyses were used to evaluate operative and long-term outcomes.
RESULTS
Residual/recurrent greater than mild-to-moderate tricuspid regurgitation at discharge was higher after cone repair compared with tricuspid valve replacement (36% vs 5%; = .010). However, at last follow-up, the risk of greater than mild-to-moderate tricuspid regurgitation was not different between groups (35% in the cone group vs 37% in the tricuspid valve replacement group; = .786). The tricuspid valve replacement group had a higher risk of tricuspid valve reoperation (37% vs 9%; = .005) and tricuspid stenosis (21% vs 0%; = .002) compared with the cone repair group. Kaplan-Meier freedom from reintervention was 97%, 91%, and 91% at 2, 4, and 6 years after cone repair, respectively, and 84%, 74%, and 68% at 2, 4, and 6 years after tricuspid valve replacement, respectively ( = .0191). At last follow-up, right ventricular function was significantly worse from baseline in the tricuspid valve replacement group ( = .0294). There were no statistical differences between age-stratified cohorts or surgeon volume in the cone repair group.
CONCLUSIONS
The cone procedure offers excellent results, with stable tricuspid valve function and low reintervention and death rates at last follow-up. The rate of greater than mild-to-moderate residual tricuspid regurgitation at discharge was higher after cone repair compared with tricuspid valve replacement, but this did not expose the patient to a higher risk of reoperation or death at last follow-up. Tricuspid valve replacement was associated with a significantly higher risk of tricuspid valve reoperation and tricuspid valve stenosis, and worse right ventricular function at last follow-up.
PubMed: 37425446
DOI: 10.1016/j.xjon.2023.03.004 -
Journal of Clinical Medicine Jul 2023The aim of the present review is to highlight the strengths and limitations of echocardiographic parameters and scores employed to predict favorable outcome in complex... (Review)
Review
Echocardiographic Parameters for Risk Prediction in Borderline Right Ventricle: Review with Special Emphasis on Pulmonary Atresia with Intact Ventricular Septum and Critical Pulmonary Stenosis.
The aim of the present review is to highlight the strengths and limitations of echocardiographic parameters and scores employed to predict favorable outcome in complex congenital heart diseases (CHDs) with borderline right ventricle (RV), with a focus on pulmonary atresia with intact ventricular septum and critical pulmonary stenosis (PAIVS/CPS). A systematic search in the National Library of Medicine using Medical Subject Headings and free-text terms including echocardiography, CHD, and scores, was performed. The search was refined by adding keywords "PAIVS/CPS", Ebstein's anomaly, and unbalanced atrioventricular septal defect with left dominance. A total of 22 studies were selected for final analysis; 12 of them were focused on parameters to predict biventricular repair (BVR)/pulmonary blood flow augmentation in PAIVS/CPS. All of these studies presented numerical (the limited sample size) and methodological limitations (retrospective design, poor definition of inclusion/exclusion criteria, variability in the definition of outcomes, differences in adopted surgical and interventional strategies). There was heterogeneity in the echocardiographic parameters employed and cut-off values proposed, with difficultly in establishing which one should be recommended. Easy scores such as TV/MV (tricuspid/mitral valve) and RV/LV (right/left ventricle) ratios were proven to have a good prognostic accuracy; however, the data were very limited (only two studies with <40 subjects). In larger studies, RV end-diastolic area and a higher degree of tricuspid regurgitation were also proven as accurate predictors of successful BVR. These measures, however, may be either operator and/or load/pressure dependent. TV Z-scores have been proposed by several authors, but old and heterogenous nomograms sources have been employed, thus producing discordant results. In summary, we provide a review of the currently available echocardiographic parameters for risk prediction in CHDs with a diminutive RV that may serve as a guide for use in clinical practice.
PubMed: 37510714
DOI: 10.3390/jcm12144599