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Brazilian Journal of Cardiovascular... Dec 2019This study aimed to evaluate Ebstein's anomaly surgical correction and its early and long-term outcomes.
OBJECTIVE
This study aimed to evaluate Ebstein's anomaly surgical correction and its early and long-term outcomes.
METHODS
A retrospective analysis of 62 consecutive patients who underwent surgical repair of Ebstein's anomaly in our institution from January 2000 to July 2016. The following long-term outcomes were evaluated: survival, reoperations, tricuspid regurgitation, and postoperative right ventricular dysfunction.
RESULTS
Valve repair was performed in 46 (74.2%) patients - 12 of them using the Da Silva cone reconstruction; tricuspid valve replacement was performed in 11 (17.7%) patients; univentricular palliation in one (1.6%) patient; and the one and a half ventricle repair in four (6.5%) patients. The patients' mean age at the time of surgery was 20.5±14.9 years, and 46.8% of them were male. The mean follow-up time was 8.8±6 years. The 30-day mortality rate was 8.06% and the one and 10-year survival rates were 91.9% both. Eleven (17.7%) of the 62 patients required late reoperation due to tricuspid regurgitation, in an average time of 7.1±4.9 years after the first procedure.
CONCLUSION
In our experience, the long-term results of the surgical treatment of Ebstein's anomaly demonstrate an acceptable survival rate and a low incidence of reinterventions.
Topics: Adolescent; Adult; Aged; Cardiac Surgical Procedures; Child; Child, Preschool; Ebstein Anomaly; Female; Humans; Infant; Kaplan-Meier Estimate; Male; Middle Aged; Postoperative Complications; Reoperation; Retrospective Studies; Severity of Illness Index; Time Factors; Treatment Outcome; Tricuspid Valve; Tricuspid Valve Insufficiency; Ventricular Dysfunction, Right; Young Adult
PubMed: 31364344
DOI: 10.21470/1678-9741-2018-0333 -
Chinese Medical Journal May 2024Ebstein's anomaly (EA) is a rare and complex congenital heart anomaly, and the effect of surgical treatment is not ideal. This study aims to introduce our experience in...
BACKGROUND
Ebstein's anomaly (EA) is a rare and complex congenital heart anomaly, and the effect of surgical treatment is not ideal. This study aims to introduce our experience in management strategies, surgical techniques, and operative indications for patients with Ebstein's anomaly.
METHODS
A retrospective study of 258 operations was performed in 253 patients by the same cardiac surgeon in The First Hospital of Tsinghua University between March 2004 and January 2020. 32 patients had previously received cardiac surgery in other hospitals. The clinical data including diagnosis, operative indications, techniques, pathological changes, and survival rates were collected and analyzed.
RESULTS
Anatomical correction was performed in 203 (78.7%) operations, 1½ ventricle repair in 38 (14.7%) operations, tricuspid valve repair only in four operations (1.6%), tricuspid valve replacement in ten (3.9%), total cavopulmonary connection (TCPC) in two (0.8%), and Glenn operation in one operation (0.4%). Reoperation was performed in five patients (2.0%) during hospitalization. Among them, tricuspid valve replacement was performed in one patient, 1½ ventricle repair in two patients, and tricuspid valve annulus reinforcement in two patients. Five patients died with an early mortality rate of 2.0%. Complete atrioventricular conduction block was complicated in one patient (0.4%). A total of 244 patients was followed up (four in the 253 patients lost) with a duration of 3.0-168.0 (87.6 ± 38.4) months. Cardiac function of 244 patients improved significantly with mean New York Heart Association (NYHA) functional class recovery from 3.5 to 1.1. The mean grade of tricuspid valve regurgitation improved from 3.6 to 1.5. Three late deaths (1.2%) occurred. The survival rates at five and ten years after surgery were 98.6% and 98.2%, respectively. Reoperation was performed in five patients (2.0%) during the follow-up period.
CONCLUSION
Based on our management strategies and operative principles and techniques, anatomical correction of EA is capable of achieving excellent long-term results, and low rates of TCPC, 1½ ventricle repair and valvular replacement.
Topics: Humans; Ebstein Anomaly; Retrospective Studies; Male; Female; Adult; Adolescent; Child; Young Adult; Child, Preschool; Tricuspid Valve; Infant; Middle Aged; Cardiac Surgical Procedures
PubMed: 38030389
DOI: 10.1097/CM9.0000000000002854 -
JTCVS Techniques Dec 2021
PubMed: 34977773
DOI: 10.1016/j.xjtc.2021.02.051 -
JACC. Case Reports Dec 2022
PubMed: 36507925
DOI: 10.1016/j.jaccas.2022.09.009 -
Interactive Cardiovascular and Thoracic... Apr 2021Our goal was to evaluate the impact of the adult congenital heart disease anatomical and physiological (ACHD AP) classification system on the surgical management of...
OBJECTIVES
Our goal was to evaluate the impact of the adult congenital heart disease anatomical and physiological (ACHD AP) classification system on the surgical management of Ebstein anomaly (EA) in adult patients.
METHODS
From February 2000 through August 2017, data of patients aged at least 16 years, who underwent primary EA surgery, were retrospectively evaluated. The cohort was divided in 2 groups according to their ACHD AP classification: the moderate EA group (IIB, IIC) and the severe EA group (IID). Survival, freedom from reoperation and freedom from occurrence of major adverse advents were estimated.
RESULTS
There were 33 patients (21 women, 12 men). Eighteen belonged to the moderate group, 15 to the severe group. There were 12 female patients (80%) in the severe group. Patients in the moderate group were younger than those in the severe group (P = 0.02): 32 ± 12 vs 44 ± 15 years old. Thirty tricuspid valve repairs and 3 replacements were performed. Repair was mainly performed in the moderate group (P = 0.02). Overall survival was 90.1 ± 5.4% at 9 months after the operation and did not change in the later follow-up period. It was 100% for patients in the moderate group and 80.0 ± 10.3% in the severe group (P = 0.07), and 75.0 ± 12.5% for female patients of in the severe group compared to 100% for the remaining patients (P = 0.025). Survival free from major adverse events, including reoperation, at 10 years was 60.0 ± 12.6% in the moderate and 38.1% ± 12.9% in the severe group (P = 0.03). No patient in the moderate group evolved to be in the severe group at late follow-up.
CONCLUSION
Adult EA patients should undergo surgery earlier when they are still in the moderate ACHD AP classification.
Topics: Cardiac Surgical Procedures; Ebstein Anomaly; Female; Heart Defects, Congenital; Humans; Male; Retrospective Studies; Treatment Outcome; Tricuspid Valve Insufficiency
PubMed: 33313770
DOI: 10.1093/icvts/ivaa294 -
Journal of Pediatric Genetics Dec 2021Trisomy 21 is considered the most common chromosomal aneuploidy, and congenital heart disease (CHD) is highly prevalent and relevant to the morbidity and mortality of...
Trisomy 21 is considered the most common chromosomal aneuploidy, and congenital heart disease (CHD) is highly prevalent and relevant to the morbidity and mortality of these patients. Ebstein anomaly (EA) is a rare CHD characterized by tricuspid valve dysplasia with inferior septal leaflet displacement. Herein, we described a patient with trisomy 21 who presented with EA and discuss the association between the two conditions based on a literature review. We conclude that the concomitant occurrence of both conditions is considered to be rare. These individuals are most frequently diagnosed during birth and childhood, and they usually have a good prognosis, as observed with our patient and is typical for EA patients in general. However, it is important to be aware that electrophysiologic anomalies may also be present.
PubMed: 34849279
DOI: 10.1055/s-0040-1714360 -
Acta Medica Indonesiana Oct 2016A 27-year-old primiparous woman with 28 weeks gestational age was admitted to our hospital with worsening shortness of breath. She was diagnosed with Ebstein's anomaly...
UNLABELLED
A 27-year-old primiparous woman with 28 weeks gestational age was admitted to our hospital with worsening shortness of breath. She was diagnosed with Ebstein's anomaly three years ago, but preferred to be left untreated. The patient was not cyanotic and her vital signs were stable. Her ECG showed incomplete RBBB and prolonged PR-interval. Blood tests revealed mild anemia. Observation of two-dimensional echo with color flow Doppler study showed Ebstein's anomaly with PFO as additional defects, EF of 57%, LV and LA dilatation, RV atrialization, severe TR, and moderate PH with RVSP of 44.3 mmHg. The patient then underwent elective sectio caesaria at 30 weeks of gestational age; both the mother and her baby were alive and were in good conditions.
PROBLEM
there was an increasing breathlessness in this patient so that there was an increasing need to take a decision for her pregnancy.Ebstein's anomaly is a complicated congenital anomaly. Medical treatment may be followed for many years in patients with mild forms of Ebstein's anomaly. Surgery should be considered if there is objective evidence of debasement such as significant enlargement of heart size, reduction of systolic function in echocardiography.
Topics: Adult; Cesarean Section; Diagnosis, Differential; Ebstein Anomaly; Echocardiography, Doppler, Color; Electrocardiography; Female; Humans; Pregnancy; Pregnancy Outcome
PubMed: 28143995
DOI: No ID Found -
Cardiology in the Young Feb 2013To assess clinical presentation, treatment, and outcome of children with Ebstein's anomaly. (Observational Study)
Observational Study
OBJECTIVES
To assess clinical presentation, treatment, and outcome of children with Ebstein's anomaly.
BACKGROUND
Data on long-term outcome of children with Ebstein's anomaly are scarce.
METHODS
Retrospective analysis of all children with Ebstein's anomaly treated between February, 1979 and January, 2009 in a single tertiary institution. Primary outcomes included patient survival and need for intervention, either cardiac surgery or catheter intervention.
RESULTS
A total of 42 patients were diagnosed with Ebstein's anomaly at a median age of 5 days ranging from 1 day to 11.7 years. Symptoms included cyanosis, heart murmur, and/or dyspnoea. Associated cardiac anomalies occurred in 90% of the patients. Average follow-up was 9.5 plus or minus 7.0 years. The overall mortality rate was 14%. Of the six patients, three died postnatally before treatment. Cardiac surgery and/or catheter-guided interventions were required in 33 patients (79%). Cardiac surgery was performed in 21 (50%) patients at a median age of 9.1 years (range 0.1-16.5 years), including biventricular repair in 13 (62%), one-and-a-half chamber repair in seven (33%), and a staged single-ventricle repair in one. Peri-operative mortality was 4%. Catheter-guided interventions consisted of device closure of an atrial septal defect in three cases and radiofrequency ablation of accessory pathways in nine patients. The estimated 10-year survival was 85.3 plus or minus 5.6%.
CONCLUSION
In children, Ebstein's anomaly is usually diagnosed in the first year of age. Even though children with Ebstein's anomaly often require an intervention, their peri-operative mortality is low and long-term survival is good. Symptomatic newborns requiring an intervention may have a worse outcome.
Topics: Cardiac Catheterization; Cardiac Surgical Procedures; Catheter Ablation; Child; Child, Preschool; Cohort Studies; Ebstein Anomaly; Heart Septal Defects, Atrial; Humans; Infant; Infant, Newborn; Retrospective Studies; Tachycardia; Treatment Outcome
PubMed: 22417890
DOI: 10.1017/S1047951112000224 -
Cureus Feb 2023Ebstein's anomaly is a congenital heart defect characterized by the displacement of the tricuspid valve, and its leaflets to be malformed. Due to the defect involving... (Review)
Review
Ebstein's anomaly is a congenital heart defect characterized by the displacement of the tricuspid valve, and its leaflets to be malformed. Due to the defect involving the tricuspid valve, there is a reverse flow of blood into the right-sided atrium, which may lead to cardiac hypertrophy and edema of the lower extremities. There is a decreased flow of blood out of the right heart due to reduced right ventricular contractility and tricuspid regurgitation. Children afflicted with this anomaly usually suffer from atrial septal defect and this is usually diagnosed before birth on a routine ultrasound scan. In neonates, cyanosis can be seen due to right-to-left atrial shunting or as a result of severe congestive heart failure. If the infant has pulmonary hypertension, cyanosis is markedly increased as there will be a limitation in pulmonary blood flow. In adults, arrhythmias, cyanosis, and heart failure are seen. The bundle of Kent leads to the formation of an electrical conduction abnormality between the right ventricle and atrium. This leads to a condition commonly known as Wolff- Parkinson-White syndrome in patients. An enlarged spherical heart is usually present on a chest X-ray. ECG changes of Ebstein's anomaly show taller than usual P waves, PR prolongation, and right bundle branch block. There can be certain neurological and extracardiac manifestations too such as hemiplegia, stroke, dysarthria, etc. During fetal life, specifically at 16 and 20 weeks of gestation, the anomaly can be diagnosed via echocardiography. Prostaglandin infusion (PGE1) is given to maintain pulmonary circulation in neonates if cyanosis is seen. In children and adults with congestive cardiac failure due to this anomaly, medical management includes digoxin, beta-blockers, diuretics, and angiotensin converting enzyme (ACE) inhibitors to improve heart failure. Surgical treatment includes valve reconstruction. In this article, we review the pathophysiology, genetics, diagnosis, management, and prognosis of Ebstein's Anomaly along with a comprehensive discussion on its genetics, neurological manifestations, extracardiac features, and current advancements in treatment.
PubMed: 36945291
DOI: 10.7759/cureus.35115 -
Journal of Thoracic Disease Jul 2018
PubMed: 30123552
DOI: 10.21037/jtd.2018.06.74