BackgroundThe incidence of Ebstein's anomaly is extremely low, and except for the Mayo Clinic, no cardiac center has reported on a sufficient number of patients. The aim of our study was to report the outcomes of Ebstein's anomaly patients treated with tricuspid valvuloplasty (TVP) or tricuspid valve replacement (TVR).

MethodsTVP or TVR was performed in 245 patients from July 2006 to April 2016. We reviewed patients' records and contacted patients via outpatient service and over the telephone.

ResultsThe mean follow-up time was 43.6 ± 32.6 months, and 224 (91.4%) patients underwent follow-up. The mean operative age was 31.2 ± 15.7 years. TVR was performed in 23 patients, and TVP was performed in 201 patients. The 30-day mortality rate was 1.3%, and the overall survival rate was 97.9% at 5 and 10 years. The early mortality rate of the TVP group was lower than that of the TVR group (0.5% vs. 8.7%, P = 0.028), and the overall mortality rate of the TVP group was lower than that of the TVR group, without statistical significance (1.0% vs. 8.7%). After propensity score matching, the rates of mortality and New York Heart Association class ≥ III were lower in the TVP group than those in the TVR group without statistical significance. Seven patients with Type B Wolff-Parkinson-White (WPW) syndrome underwent one-stage surgery, and arrhythmias disappeared. Six patients suffered from episodes of left ventricular outflow tract obstruction (LVOTO) during surgery. Severe LVOTO could be treated with reoperation of the atrialized right ventricle.

ConclusionsEbstein's anomaly patients treated with TVP or TVR can experience optimal outcomes with midterm follow-up. However, TVP should be the first-choice treatment. Optimal outcomes can be obtained from one-stage operation in patients with Type B WPW syndrome. Severe LVOTO during surgery might be related to improper operation of the atrialized right ventricle.

Adolescent ; Adult ; Aged ; Cardiac Surgical Procedures ; methods ; Child ; Ebstein Anomaly ; surgery ; Female ; Humans ; Male ; Middle Aged ; Reconstructive Surgical Procedures ; methods ; Retrospective Studies ; Tricuspid Valve ; surgery ; Tricuspid Valve Insufficiency ; surgery ; Young Adult

PURPOSE: Spontaneous neonatal gastric perforation is a rare but fatal disease with unclear etiology. In this study, we reviewed its clinical manifestations, outcomes, and discussed the etiology and prognostic factors. METHODS: There were 12 neonates with gastric perforation in our hospital from 1989 to 2015. Their medical records were reviewed retrospectively including birth record, associated disease, site and size of perforation, type of surgical management, clinical outcome. Also, the prognostic factors were analyzed. RESULTS: The median gestational age and birth weight was 32 weeks (range, 26-43 weeks; preterm birth rate, 66.7%) and 1,883 g (range, 470-4,400 g), respectively. Five patients had associated gastrointestinal anomalies including esophageal atresia and tracheoesophageal fistula (two patients), midgut volvulus, non-rotation and microcolon, and meconium plug syndrome. The median age at surgery was six days after birth (range, 2-13 days), and the median weight at surgery was 1,620 g (range, 510-3,240 g). Upper third part of stomach was the most frequently involved location of perforation. The size of perforation varied from pin point to involving the whole greater curvature. Primary repairs were done in seven cases, and in five cases, resections of necrotic portion were needed. Mortality rate was 33.3% (n=4), morbidity (re-operation) rate was 16.7% (n=2). The causes of death were sepsis (n=3), and heart failure from Ebstein anomaly (n=1). The median hospital stay was 92.5 days (range, 1-176 days). The factors mentioned as prognostic factors in previous studies showed no significant relations to the mortality and morbidity in our study. CONCLUSION: There were improvements of outcomes in patients with large size perforation. As previous studies, we assume these improvements were possible due to the improvements of critical care medicine. Given that rare incidence, a multi-center study can help us get a better understanding of this disease, and a better outcome.
Birth Certificates ; Birth Weight ; Cause of Death ; Critical Care ; Ebstein Anomaly ; Esophageal Atresia ; Gestational Age ; Heart Failure ; Humans ; Incidence ; Infant, Newborn ; Intestinal Volvulus ; Length of Stay ; Meconium ; Medical Records ; Mortality ; Parturition ; Premature Birth ; Retrospective Studies ; Sepsis ; Stomach ; Tracheoesophageal Fistula

In patients with Ebstein's anomaly, the localization of accessory pathways may be impeded by abnormal local electrograms recorded along the atrialized right ventricle and by the presence of multiple accessory pathways. We report a case of 50-year-old man diagnosed with Ebstein's anomaly with Wolff-Parkinson-White syndrome who presented with recurrent palpitations. He was referred to our institution for radiofrequency catheter ablation of paroxysmal supraventricular tachycardia. Transthoracic echocardiography revealed the tricuspid valve displaced into the right ventricle, consistent with Ebstein's anomaly. The electrophysiology study showed a right posterolateral accessory pathway. The optimal ablation site was located not in the atrioventricular line of the atrialized ventricular portion, but in the original atrioventricular line.
Accessory Atrioventricular Bundle ; Catheter Ablation ; Ebstein Anomaly* ; Echocardiography ; Electrophysiology ; Heart Ventricles ; Humans ; Middle Aged ; Tachycardia, Supraventricular ; Tricuspid Valve ; Wolff-Parkinson-White Syndrome

The objectives of this study were to assess the clinical outcomes of adults with Ebstein Anomaly (EA) according to their treatment modalities. All adult EA patients diagnosed between October 1994 and October 2014 were retrospectively evaluated by medical record review. Total 60 patients were categorized into 3 groups according to their treatment strategy, i.e. non-operative treatment (Group I, n = 23), immediate operative treatment (Group II, n = 27), and delayed operative treatment (Group III, n = 10). A composite of major adverse cardiac and cerebrovascular events (MACCE) and factors associated with MACCE were assessed in each treatment group. MACCE occurred in 13.0% patients in Group I, 55.6% patients in Group II and 50% in Group III (P = 0.006). Event free survivals at 5 years were 90% in Group I, 52.7% in Group II, 50.0% in Group III (P = 0.036). Post-operatively, most patients showed improvement on clinical symptoms. However, event free survival rate was lower in patients with operation compared to those with non-operative treatment (58.7% vs. 90.9%; P = 0.007). Major arrhythmic event occurred more frequently even after surgical ablation (50.0% vs. 20.0%; P = 0.034). Re-operation was more frequent in patients underwent delayed surgery compared to those with immediate surgery (50.0% vs. 18.5%; P = 0.001). Current guideline to decide patient's treatment strategy appeared to be appropriate in adult patients with EA. However, surgical ablation for arrhythmia was not enough so that concomitant medical treatment should be considered. Therefore, attentive risk stratification and cautious decision of treatment strategy by experienced cardiac surgeon are believed to improve clinical outcome.
Adult* ; Arrhythmias, Cardiac ; Disease-Free Survival ; Ebstein Anomaly* ; Humans ; Medical Records ; Retrospective Studies

The management of recurrent tricuspid regurgitation after tricuspid valve repair in patients with Ebstein anomaly is difficult, and tricuspid valve replacement is most commonly performed in such patients. We report two cases of recurrent tricuspid regurgitation in patients with Ebstein anomaly that were successfully re-repaired using the cone technique. The cone repair technique is a useful surgical method for reconstructing a competent tricuspid valve, and can be applied in patients who have undergone previous tricuspid valve repair.
Ebstein Anomaly* ; Heart Defects, Congenital ; Humans ; Tricuspid Valve Insufficiency ; Tricuspid Valve*

BACKGROUND AND OBJECTIVES: The aim of the study is to evaluate the long-term results after a surgical repair of Ebstein's anomaly. SUBJECTS AND METHODS: Forty-eight patients with Ebstein's anomaly who underwent open heart surgery between 1982 and 2013 were included. Median age at operation was 5.6 years (1 day-42.1 years). Forty-five patients (93.7%) demonstrated tricuspid valve (TV) regurgitation of less than moderate degree. When the patients were divided according to Carpentier's classification, types A, B, C, and D were 11, 21, 12, and 4 patients, respectively. Regarding the type of surgical treatment, bi-ventricular repair (n=38), one-and-a half ventricular repair (n=5), and single ventricle palliation (n=5) were performed. Of 38 patients who underwent a bi-ventricular repair, TV repairs were performed by Danielson's technique (n=20), Carpentier's technique (n=11), Cone repair (n=4), and TV annuloplasty (n=1). Two patients underwent TV replacement. Surgical treatment strategies were different according to Carpentier's types (p<0.001) and patient's age (p=0.022). RESULTS: There were 2 in-hospital mortalities (4.2%; 1 neonate and 1 infant) and 2 late mortalities during follow-up. Freedom from recurrent TV regurgitation rates at 5, 10, and 15 years were 88.6%, 66.3%, 52.7%, respectively. TV regurgitation recurrence did not differ according to surgical method (p=0.800). Survival rates at 5, 10, and 20 years were 95.8%, 95.8%, and 85.6%, respectively, and freedom from reoperation rates at 5, 10, and 15 years were 85.9%, 68.0%, and 55.8%, respectively. CONCLUSION: Surgical treatment strategies were decided according to Carpentier's type and patient's age. Overall survival and freedom from reoperation rates at 10 years were 95.8% and 68.0%, respectively. Approximately 25% of patients required a second operation for TV during the follow-up.
Classification ; Cyanosis ; Ebstein Anomaly* ; Follow-Up Studies ; Freedom ; Heart Defects, Congenital ; Hospital Mortality ; Humans ; Infant, Newborn ; Methods ; Mortality ; Recurrence ; Reoperation ; Survival Rate ; Thoracic Surgery ; Tricuspid Valve

Twenty-six-year-old Ebstein's anomaly patient, who had failed both biventricular and one-and-a-half repair, underwent right ventricle exclusion and Fontan operation. She completed pregnancy and delivery owing to the excellent long-term clinical course. Although the caesarean section was performed due to symptoms of heart failure on the gestational age of 32+6 weeks, preterm delivery was agreeable with neonatology support. In Korea, there has not yet been a case of pregnancy and delivery of functional single ventricle patient because most patients have been discouraged from getting pregnant. However, functional single ventricle patient can endure pregnancy and delivery, if valve function and ventricular contractility, status of Fontan pathway and absence of arrhythmia predict favorable outcome as presented in this case. Although the patient maintained her pregnancy without anticoagulation owing to laminar flow in the Fontan pathway and absence of thromboembolic event, anticoagulation should be considered, weighing the benefits and risks during the pregnancy.
Arrhythmias, Cardiac ; Cesarean Section ; Delivery, Obstetric ; Ebstein Anomaly ; Female ; Fontan Procedure* ; Gestational Age ; Heart Failure ; Heart Ventricles* ; Humans ; Korea ; Neonatology ; Pregnancy* ; Risk Assessment

Twenty-six-year-old Ebstein's anomaly patient, who had failed both biventricular and one-and-a-half repair, underwent right ventricle exclusion and Fontan operation. She completed pregnancy and delivery owing to the excellent long-term clinical course. Although the caesarean section was performed due to symptoms of heart failure on the gestational age of 32+6 weeks, preterm delivery was agreeable with neonatology support. In Korea, there has not yet been a case of pregnancy and delivery of functional single ventricle patient because most patients have been discouraged from getting pregnant. However, functional single ventricle patient can endure pregnancy and delivery, if valve function and ventricular contractility, status of Fontan pathway and absence of arrhythmia predict favorable outcome as presented in this case. Although the patient maintained her pregnancy without anticoagulation owing to laminar flow in the Fontan pathway and absence of thromboembolic event, anticoagulation should be considered, weighing the benefits and risks during the pregnancy.
Arrhythmias, Cardiac ; Cesarean Section ; Delivery, Obstetric ; Ebstein Anomaly ; Female ; Fontan Procedure* ; Gestational Age ; Heart Failure ; Heart Ventricles* ; Humans ; Korea ; Neonatology ; Pregnancy* ; Risk Assessment

OBJECTIVE: To summarize the outcome of tricuspid valve replacement.
 METHODS: A total of 28 patients (15 males and 13 females) underwent tricuspid valve replacement from March 2000 to February 2015 in the First Affiliated Hospital of Zhengzhou University were recruited. Among them, 16 patients were Ebstein's anomaly, 7 had rheumatic valve heart disease, 3 and 2 suffered from infective endocarditis and degenerative tricuspid lesions, respectively.
 RESULTS: One patient died of multiple organ failure. Four patients were implanted permanent cardiac pacemaker because of third degree atrioventricular block occurring in the 5th day (2 patients) and in the 9th day (2 patients) after the operation, respectively. Twenty-seven patients were followed up from 1 month to 15 years. The prosthetic valves and permanent pacemakers worked well.
 CONCLUSION Third degree of atrioventricular block, mostly appearing in early postoperative period, is the most common and severe complication of tricuspid valve replacement. The key point for prevention of damage is to accurately identify the anatomical relationship among the tricuspid valve, atrioventricular node, and conduction bundle.
Ebstein Anomaly ; surgery ; Female ; Heart Valve Diseases ; surgery ; Heart Valve Prosthesis ; Heart Valve Prosthesis Implantation ; Humans ; Male ; Pacemaker, Artificial ; Rheumatic Heart Disease ; surgery ; Tricuspid Valve ; pathology ; surgery

Ebstein malformation is a congenital heart disease characterized pathologically by displacement of the septal leaflet of the tricuspid valve towards the apex of the right ventricle of the heart. Hypoplasia, dysfunction of the right ventricle and tricuspid regurgitation cause an increased volume load of the right heart and result in the clinical manifestations of chest tightness, shortness of breath and fatigue after activities, palpitation, cyanosis and heart failure. We report a case of Ebstein's anomaly with refractory right heart failure and leg ulcers.
Ebstein Anomaly ; Heart Failure ; Heart Ventricles ; physiopathology ; Humans ; Leg Ulcer ; Tricuspid Valve Insufficiency