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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 -
JACC. Case Reports May 2020Tropical endomyocardial fibrosis is a common cause of restrictive cardiomyopathy worldwide, but is relatively rare in developed countries. We present a case of tropical...
Tropical endomyocardial fibrosis is a common cause of restrictive cardiomyopathy worldwide, but is relatively rare in developed countries. We present a case of tropical endomyocardial fibrosis with right ventricular involvement initially mistaken as Ebstein's anomaly. We highlight the need for timely and accurate diagnosis to ensure appropriate management. ().
PubMed: 34317354
DOI: 10.1016/j.jaccas.2020.02.020 -
World Journal For Pediatric &... Nov 2020Ebstein anomaly has a breadth of presentations, including "typical" and "atypical," and can be confused with congenital tricuspid dysplasia. We summarize how to... (Review)
Review
Ebstein anomaly has a breadth of presentations, including "typical" and "atypical," and can be confused with congenital tricuspid dysplasia. We summarize how to differentiate within this spectrum of disease. Both typical and atypical Ebstein have an underlying failure of delamination, but atypical Ebstein does not have ≥8mm/m apical septal leaflet displacement. In congenital tricuspid dysplasia, delamination is normal, while the leaflets and subvalvar apparatus are abnormal. To summarize, the sine qua non feature of Ebstein anomaly, present in both typical and atypical, is the failure of delamination. These are distinct from congenital tricuspid valve dysplasia in which the pathology is in the leaflet itself.
Topics: Cardiac Surgical Procedures; Child; Ebstein Anomaly; Echocardiography; Humans; Tricuspid Valve
PubMed: 33164686
DOI: 10.1177/2150135120949235 -
Veterinary Research Forum : An... 2022A one and a half years old male French bulldog weighing 9.50 kg was presented with the history of inappetence, lethargy, abdominal distension and exercise intolerance...
A one and a half years old male French bulldog weighing 9.50 kg was presented with the history of inappetence, lethargy, abdominal distension and exercise intolerance since last 2 days. The physical examination was done which revealed normal physiological parameters including temperature, mucus membrane color and capillary refill time except palpable precordial thrills, jugular distension on palpation, tachycardia and systolic murmurs on auscultation. Electrocardiography (ECG) was done which depicted ectopic foci with atrioventricular junctional tachycardia and right ventricular enlargement involving very small inverted P waves, deep S waves in leads I, II, III and augmented vector foot (aVF) and splintered QRS complexes. The dog was undergone chest radiography that revealed right atrial enlargement, increased sternal contact of heart on lateral view and a bulge at 9:00 o'clock to 11:00 o'clock depicted right atrial enlargement on dorso-ventral view. Lastly, echocardiography was done to arrive at a diagnosis confirming the Ebstein's anomaly as a form of tricuspid valve dysplasia including apical displacement of tricuspid valve leaflets, division of right ventricle into atrialized and functional portions, increased displacement index, increased apex-mitral annulus to apex-tricuspid annulus ratio, severe right atrial dilatation and tricuspid regurgitation. The dog was medically treated with diuretics, angiotensin converting enzyme inhibitors and inotropes and the owner was advised to put the dog on low sodium diet for 2 weeks. The dog has resolved clinical signs of right sided heart affection; but, suddenly collapsed at home. The owner denied for the necropsy of dog.
PubMed: 36686865
DOI: 10.30466/vrf.2022.550981.3425 -
Cardiology Clinics May 2020Survivorship into adulthood of patients with congenital heart disease is due to improvements in prenatal detection, novel surgeries, and specialized adult congenital... (Review)
Review
Survivorship into adulthood of patients with congenital heart disease is due to improvements in prenatal detection, novel surgeries, and specialized adult congenital heart disease care. As patients survive further into adulthood, long-term complications of congenital and repaired physiology have been more clearly elucidated. The overall mortality of patients with adult congenital heart disease with heart failure is around 4%. Congenital malformations, palliations, residual defects, and resultant physiology impact the right ventricle. This relationship influences morbidity and mortality. For this discussion, focus on atrial septal defects, Ebstein anomaly, Tetralogy of Fallot, transposition of the great vessels, and single right ventricle physiology.
Topics: Heart Defects, Congenital; Heart Failure; Heart Ventricles; Humans; Ventricular Function, Right
PubMed: 32284100
DOI: 10.1016/j.ccl.2020.02.002 -
The Journal of Thoracic and... Mar 2021
Topics: Ebstein Anomaly; Humans
PubMed: 33431212
DOI: 10.1016/j.jtcvs.2020.12.004 -
Circulation. Cardiovascular Imaging Mar 2021
Topics: Ebstein Anomaly; Exercise Tolerance; Fibrosis; Heart Ventricles; Humans; Myocardium
PubMed: 33722058
DOI: 10.1161/CIRCIMAGING.121.012285 -
Journal of Echocardiography Mar 2023
Topics: Humans; Ebstein Anomaly; Heart Ventricles
PubMed: 34436755
DOI: 10.1007/s12574-021-00548-3 -
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