Atypical pompholyx presentation of secondary Staphyloccoccus and Klebsiella infections in a patient with premorbid Ebstein anomaly: A case report

Rationale: Pompholyx refers to pruritic vesicles or bullous rash that mainly distribute on the palms and lateral surfaces of the fingers. It is less common among Asians, and in a severe condition, secondary bacterial infection of pompholyx can happen and result in pain, swelling and pustules. Patient concerns: A 15-year-old girl complained of progressive wound and small bumps containing yellowish pus and crusts on her hands and feet for over 6 months and worsened in the last month before admission. She also had Ebstein anomaly. Diagnosis: Atypical pompholyx with secondary Staphylococcus and Klebsiella infections. Interventions: Wound care with wet dressing and applying moisturizer on crusts, application of antibiotics for Gram positive and negative bacteria and giving nutritional support with reckoning of proper calories. Outcomes: Skin lesions were completely healed and the patient was discharged after 10 days of hospitalization. Lessons: Atypical manifestation of pompholyx makes it hard to diagnose. The diagnosis can be confirmed with meticulous history-taking and physical examination. Wound caring and controlling of the infection should be done to earn an optimal outcome.

Anomalía de Ebstein en recién nacido / Ebstein's anomaly in newborn

Introducción: La anomalía de Ebstein es una rara malformación con presentación sintomática respiratoria leve a grave a causa de defectos cardiacos congénitos manifestados durante la niñez. Objetivo: Exponer el caso en el cual el diagnóstico oportuno de esta enfermedad permitió el tratamiento integral del paciente. Presentación del caso: Recién nacido a término, quien a las siete horas de su nacimiento presentó saturación de oxígeno disminuida, examen físico sin cianosis, soplo pansistólico en la auscultación y cardiomegalia detectada por rayos X de tórax. Se realizó ecocardiograma que permitió diagnosticar al bebé como portador de la enfermedad de Ebstein con manifestaciones leves. Una vez que tiene mejoría clínica, se le proporcionó el alta médica con indicación de valoración por cardiología pediátrica. Conclusiones: La anomalía de Ebstein puede comenzar en el período neonatal con sintomatología de severidad variable. La sospecha clínica permite la confirmación diagnóstica y el seguimiento estrecho, sobre todo en casos severos(AU)

Introduction: Ebstein's anomaly is a rare malformation with mild to severe respiratory symptomatic presentation due to congenital heart defects manifested during childhood. Objective: To present the case in which the timely diagnosis of this disease allowed the comprehensive treatment of the patient. Case presentation: Full-term newborn, who at seven hours of birth presented decreased oxygen saturation, physical examination without cyanosis, pansystolic murmur in auscultation and cardiomegaly detected by chest X-rays. An echocardiogram was performed to diagnose the baby as a carrier of Ebstein's disease with mild manifestations. Once he had clinical improvement, he was discharged with an indication for assessment by pediatric cardiology. Conclusions: Ebstein's anomaly may begin in the neonatal period with symptoms of variable severity. Clinical suspicion allows for diagnostic confirmation and close follow-up, especially in severe cases(AU)

Neonatal Ebstein's anomaly with type B Wolff-Parkinson-White syndrome: a case report / 中华围产医学杂志

This paper reported a male newborn with Ebstein's anomaly complicated by type B Wolff-Parkinson-White syndrome. The baby was delivered at 39 weeks of gestation and diagnosed as Ebstein's anomaly complicated by type B Wolff-Parkinson-White syndrome 2 d after birth. He was improved and discharged after conservative management in the Department of Internal Medicine. No abnormality was found by fetal echocardiography at 20 weeks of gestation, while supraventricular tachycardia was diagnosed by M-mode ultrasonography at 38 weeks of gestation due to rapid fetal heart rate. In addition to hemodynamic changes,the abnormal cardiac structure in patients with Ebstein's anomaly may also lead to tachyarrhythmia. Therefore, in fetuses diagnosed with supraventricular tachycardia by prenatal ultrasound or children with type B Wolff-Parkinson-White syndrome detected by electrocardiogram after birth, tricuspid valve should be carefully scanned under echocardiography to avoid the missed diagnosis of Ebstein's anomaly.

Rare diagnostics of Ebstein anomaly in an adult patient in Northern Rio de Janeiro / Diagnóstico raro da anomalia de Ebstein no norte do Rio de Janeiro em um paciente adulto

Ebstein anomaly is the fourth most frequent cyanotic heart disease in the neonatal period, and its clinical course is slow compared to other diseases, which in turn results in late diagnosis of this condition. We present a case of a 37-year-old man who complained of palpitation for about 5 years, twice a week, at rest, with prolonged duration and low systemic output. Auscultation revealed irregular heart rhythm characterized by extrasystoles with a tricuspid systolic murmur grade 5+/6 and worsening on inspiration. Clinical spectrum is variable and depends on the severity of defects and associated anatomical changes. In patients who have reached adulthood, symptoms usually present as progressive cyanosis, exercise intolerance, right heart failure, and arrhythmias. In Brazil, identification of this congenital disease usually occurs in a more adult phase, affecting the prognosis and survival of patients. (AU)

Anomalia de Ebstein é a quarta cardiopatia cianótica com maior frequência no período neonatal, cuja evolução clínica é lenta em comparação com outros tipos de doenças, o que, por sua vez, resulta no diagnóstico tardio. Apresentamos o caso de um homem de 37 anos, com queixa de palpitações por cerca de cinco anos, duas vezes por semana, em repouso, com duração prolongada e com baixo débito sistêmico. A ausculta revelou ritmo cardíaco irregular por extra-sístoles com sopro sistólico na tricúspide, 5 + / 6 piora na inspiração. O espectro clínico é variável e ocorre de acordo com a gravidade dos defeitos e alterações anatômicas associadas. Pacientes que atingiram a idade adulta, os sintomas geralmente se apresentam como cianose progressiva, intolerância ao exercício, insuficiência cardíaca direita e arritmias. A realidade brasileira é que a identificação dessa doença congênita persiste em uma fase adulta, afetando o prognóstico e a sobrevida desses pacientes. (AU)

Clinical analysis of outcomes of different surgery methods for Ebstein anomaly in a single center / 中国胸心血管外科临床杂志

@#Objective    To investigate the indications and clinical effects of tricuspid cone reconstruction and tricuspid valve replacement in the treatment of downward displacement of tricuspid valve (Ebstein anomaly). Methods    The clinical data of 22 patients with Ebstein anomaly who underwent surgical treatment in our hospital from January 2013 to March 2020 were collected. There were 7 males and 15 females, aged 4-56 (33.68±17.78) years. The patients were divided into two groups according to different surgical methods: a tricuspid cone reconstruction group (tricuspid valvuloplasty group, n=12) and a tricuspid valve replacement group (n=10). The curative effect of the two operative methods were compared. Results    Twenty-two patients underwent surgical treatment under general anesthesia and cardiopulmonary bypass. One patient died of severe low cardiac output syndrome during operation. Downward displacement of tricuspid septum was found in 22 patients, and downward displacement of tricuspid septum and posterior septum in 21 patients, downward displacement of tricuspid in 8 patients. Twelve patients were treated with tricuspid  valvuloplasty, 10 patients with tricuspid valve replacement, and patients with other intracardiac structural malformations were treated at the same time. Postoperative cardiac doppler ultrasound indicated no tricuspid regurgitation in 9 patients, mild regurgitation in 8 patients, and moderate regurgitation in 4 patients. No perivalvular leakage occurred in all patients undergoing valve replacement. Four patients developed degree Ⅲ atrioventricular block after operation, among whom 3 patients recovered to sinus rhythm and 1 patient implanted the permanent pacemaker. Twenty-one patients were cured and discharged after successful operation, and were followed up for 3 to 78 months, with a follow-up rate of 100.0%. During the follow-up period, there was no severe tricuspid regurgitation in tricuspid valvuloplasty group, but mild or moderate regurgitation was found. After tricuspid valve replacement, only 1 patient had mild regurgitation, and the rest patients had no valve regurgitation. Conclusion    Tricuspid valvuloplasty and tricuspid valve replacement are effective in the treatment of Ebstein anomaly, and the tricuspid regurgitation is less severe after tricuspid valve replacement operation. The operation method should be selected according to the different anatomical characteristics and condition of tricuspid valve. The combined intracardiac malformation or arrhythmia can be dealt with simultaneously.

Reoperative strategy and mid- to long-term surgical outcomes of Ebstein anomaly / 中国胸心血管外科临床杂志

@#Objective    To analyze the Ebstein anomaly's reoperative strategy and mid- to long-term results. Methods    We retrospectively reviewed the clinical data of 23 patients who diagnosed with Ebstein anomaly and underwent reoperation for tricuspid valve insufficiency between July 2002 and July 2017 in Fuwai Hospital. There were 9 (39.1%) males and 14 (60.9%) females, with a median age of 28.0 (19.0, 45.0) years. Results    Among the 23 patients, 8 (34.8%) underwent tricuspid valvuloplasty and 15 (65.2%) underwent tricuspid valve replacement. The rate of valvuloplasty was 16.7% before 2012, and 54.5% after 2012 (P=0.089) as Cone reconstruction procedure was used. In the valvuloplasty cohort, 3 (37.5%) patients were treated with Danielson or Carpentier technique, and 5 (62.5%) patients were treated with Cone reconstruction procedure. There was no operation-related death. Early complications occurred in 3 (37.5%) patients. The median follow-up was 6.9 years (range, 3.0-15.1 years), and no adverse cardiac events occurred. In the patients with valve replacement, 7 (46.7%) received mechanical prosthesis and 8 (53.3%) received bio-prosthesis. There was no operation-related death. And early complications were observed in 3 (20.0%) patients. The median follow-up was 6.5 years (range, 2.5-15.3 years). One (6.3%) patient died and 4 (26.7%) had long-term complications during the follow-up period. Conclusion    The mid- to long-term outcomes are convincing in patients who undergo the second operation due to recurrent tricuspid regurgitation of Ebstein anomaly. A low incidence of reoperation is observed. Cone reconstruction procedure provides possibilities of second tricuspid valvuloplasty, and this technique can reduce the rate of tricuspid valve replacement in the second operation. Tricuspid valve replacement is still an alternative method for the treatment of recurrent tricuspid regurgitation in patients with Ebstein anomaly. The bioprosthetic prosthesis may be a better choice than mechanical prosthesis.

Caso clínico: adulto con anomalía de Ebstein / Clinical case: adult with Ebstein anomaly

Introducción: la anomalía de Ebstein se caracteriza por la implantación baja de las valvas septal y posterior de la tricúspide; por lo tanto, la aurícula derecha es amplia y su ventrículo es pequeño. Al ser una patología bastante rara en nuestro medio se encuentra subdiagnosticada y no recibe el tratamiento oportuno. Por ende, la importancia de conocerla para no retrasar su manejo clínico quirúrgico.Caso clínico: paciente de 46 años de edad, masculino, con antecedente de hipoacusia congénita, presentó disnea (clase funcional III) más palpitaciones y dolor torácico atípico hace 1 año aproximadamente. Al examen físico evidenció soplo holosistólico en foco tricuspídeo y uso moderado de musculatura accesoria. Al ecocardiograma transesofágico, se observó arritmia auricular más implante bajo de las valvas de la tricúspide y se inició tratamiento clínico más ablación del flutter. En la evolución presentó mejoría de la sintomatología y dependiendo del deterioro de la clase funcional, tratamiento quirúrgico Conclusiones: en el presente caso se ajustó tratamiento clínico y ablación del flutter de acuerdo a sintomatología del paciente, mostrando mejoría (AU);

Introduction: Ebstein's anomaly is characterized by low implantation of the septal and posterior tricuspid leaflets; therefore, the right atrium is wide and its ventricle is small. Being a rather rare pathology in our environment, it is underdiagnosed and does not receive the appropriate treatment. Therefore, it is important to know it to avoid a delay in its clinical surgical management.Clinical case: The patient is 46-year-old male, with a history of congenital hearing loss, who presented dyspnea (functional class III) plus palpitations and atypical chest pain approximately 1 year ago. In the physical examination, he revealed a holosystolic murmur in the tricuspid focus and moderate use of accessory muscles. A transesophageal echocardiogram revealed atrial arrhythmia plus implantation of the tricuspid leaflets and it was started a clinical treatment plus flutter ablation. In the evolution he presented improvement of the symptoms and depending on the deterioration of the functional class, surgical treatment.Conclusions: in this case, clinical treatment and flutter ablation were adjusted according to the patient's symptoms, showing improvement (AU);

Válvula tricúspide displásica (Ebstein-like) / Tricuspid valve dysplasia (Ebstein-like)

Resumen La anomalía de Ebstein es una malformación caracterizada por el desplazamiento apical de las inserciones de las valvas septal y posterior de la válvula tricúspide, así como del anillo funcional tricúspide y por la atrialización del ventrículo derecho. Se han encontrado casos durante cirugías que no han cumplido con estos criterios, y que, por tanto, se han clasificado como "anomalías Ebstein-like". Algunos de ellos han sido descritos anatómicamente como válvulas displásicas. Se presenta el caso de una mujer de 20 años que presentó disnea clase III de la Asociación del Corazón de Nueva York y dolor punzante en el pecho, que se clasificó como una posible anomalía de Ebstein por ecocardiografía; sin embargo, los hallazgos intraoperatorios fueron compatibles con una válvula tricúspide displásica con dilatación del anillo funcional. Este caso destaca la importancia del uso adecuado de la ecocardiografía, así como la necesidad de estudios prospectivos sobre los casos de Ebstein-like.

Abstract The Ebstein anomaly is a malformation characterised by the apical displacement of the insertions of the septal valve and the posterior tricuspid valve, displaced tricuspid functional ring, and atrialisation of the right ventricle. Cases have been found during surgery that have complied with these criteria, and have therefore been classified as "Ebstein-like anomalies". Some of them have been described anatomically as valve dysplasia. The case is presented of a 20 year-old female that presented with dyspnoea Class III of the New York Heart Association, and a stabbing pain in the chest. This was classified as a possible Ebstein anomaly by cardiac ultrasound. However the findings during the surgery were compatible with tricuspid valve dysplasia with dilation of the functional ring. This case highlights the importance of the appropriate use of cardiac ultrasound, as well as the need to carry out prospective studies on Ebstein-like cases.

Consideraciones útiles para el asesoramiento genético de pacientes con anomalía de Ebstein / Useful considerations for the genetic advice of patients with Ebstein anomaly

La anomalía de Ebstein es una rara enfermedad que consiste en un espectro variable de anomalías, resultado de una implantación anormal de la válvula tricúspide en el ventrículo derecho; por tal razón, es considerada una afección grave e incurable que origina una tendencia a la terminación voluntaria del embarazo como opción reproductiva en el marco del asesoramiento genético prenatal. Los adelantos en el diagnóstico y control clinicoquirúrgico de esta enfermedad han garantizado el incremento de la supervivencia y una mejor calidad de vida en estos pacientes. Además, han permitido ajustar factores clínicos que implican su evolución y pronóstico. Se realizan algunas valoraciones, a fin de proveer los argumentos necesarios que permitan a la pareja elegir un curso de acción apropiado con vistas a los riesgos y objetivos familiares basados en los principios éticos del asesoramiento genético.

Ebstein anomaly is a strange disease that consists on a variable spectrum of anomalies, due to an abnormal installation of the tricuspid valve in the right ventricle; reason why, it is considered a serious and incurable disorder that originates a tendency to the voluntary termination of pregnancy as reproductive option in the mark of prenatal genetic advice. The advances in the diagnosis and clinical surgical control of this disease have guaranteed the increment of survival and a better life quality in these patients. Also, they have allowed to adjust clinical factors that involve its clinical course and prognosis. Some valuations are carried out, in order to provide the necessary arguments that allow the couple to choose an appropriate action course aimed at the risks and family objectives based on the ethical principles of the genetic advice.

Clinical outcome of tricuspid valve replacement in children aged no more than 14 years / 中国胸心血管外科临床杂志

@#Objective    To summarize the clinical characteristics and outcome of tricuspid valve replacement (TVR) in children aged no more than 14 years, and to discuss the selection of prosthesis. Methods    From September 2002 to August 2019, 14 patients aged no more than 14 years who received TVR were included in our study. There were 9 males and 5 females, with a mean age of 9.8±4.3 years. Results    Mechanical prosthesis was implanted in 8 patients, and bioprosthesis in 6 patients. The mean cardiopulmonary time and aortic-clamp time was 170.3±109.8 min and 95.1±63.1 min, respectively. The mortality within 30 days after surgery was 21.4% (3/14), and all 3 patients died of severe low cardiac output syndrome. Eleven patients were followed up for 34-199 (100.1±57.4) months. During the follow-up, mechanical prosthesis dysfunction occurred in 3 patients, 2 of whom received secondary TVR. One patient died during the follow-up. Conclusion    The bioprosthesis is the first choice for TVR in children. Some long-term complications may occur after TVR, and close follow-up and timely intervention are needed.

Early and long-term outcomes of surgical treatment of ebstein's anomaly

Abstract 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.

Transvenous Low Septal Permanent Pacing for a Patient with Ebstein’s Anomaly – A Case Report

Patients with Ebstein’s anomaly can present with bradyarrhythmias at any age. In view of the abnormal tricuspid valve anatomy associated with this condition, pacing these individuals can present with various challenges. We present a patient with Ebstein’s anomaly

Anesthetic management for patient with severe cyanosis following bioprosthetic valve stenosis / Manejo anestésico de paciente com cianose grave após estenose de valva bioprotética

Abstract We presented a 39-year-old female patient with life-threatening hypoxemia after tricuspid valve replacement because of Ebstein's anomaly. And the severe cyanosis is due to bioprosthetic valve stenosis and atrial septal defect. Anesthetic management of a patient with severe obstructive prosthetic valve dysfunction can be challenging. Similar considerations should be given to patients with Ebstein's anomaly to maintain the pressure equalized between the right and left atrial. Transesophageal echocardiography and cerebral oxygen saturation provided real time information in perioperative care.

Resumo Apresentamos o caso de uma paciente de 39 anos, com hipoxemia em risco de vida após a substituição da valva tricúspide devido à anomalia de Ebstein e cianose grave devido à estenose de valva bioprotética e comunicação interatrial. O manejo anestésico de um paciente com disfunção obstrutiva grave de prótese valvar pode ser um desafio. Os pacientes com anomalia de Ebstein também precisam de atenção especial para manter a pressão equalizada entre o átrio direito e o esquerdo. A ecocardiografia transesofágica e a saturação cerebral de oxigênio forneceram informações em tempo real nos cuidados perioperatórios.

Electrocardiography findings and clinical presentation in Ebstein's anomaly

Ebstein's anomaly is a congenital malformation characterised by tricuspid valve pathology with right heart enlargement. Cases of Ebstein's anomaly can vary widely in severity, anatomy and presentation. In this article, we presented three cases of Ebstein's anomaly and discussed the presentation as well as electrocardiographic (ECG) changes. Patients may first present to their primary care physicians with cardiac symptoms such as reduced effort tolerance together with an abnormal ECG. ECG changes suggestive of right heart enlargement are important in the initial consideration and eventual formal diagnosis of the condition.

Ebstein's anomaly: "The one and a half ventricle heart"

Abstract Objective: Ebstein's anomaly remains a relatively ignored disease. Lying in the 'No Man's land' between congenital and valve surgeons, it largely remains inadequately studied. We report our short-term results of treating it as a 'one and a half ventricle heart' and propose that the true tricuspid annulus (TTA) 'Z' score be used as an objective criterion for estimation of 'functional' right ventricle (RV). Methods: 22 consecutive patients undergoing surgery for Ebstein's anomaly were studied. Echocardiography was performed to assess the type and severity of the disease, tricuspid annular dimension and its 'Z' score. Patients were operated by a modification of the cone repair, with addition of annuloplasty, bidirectional cavopulmonary shunt (BCPS) and right reduction atrioplasty to provide a comprehensive repair. TTA 'Z' score was correlated later with postplication indexed residual RV volume. Results: There was one (4.5%) early and no late postoperative death. There was a significant reduction in tricuspid regurgitation grading (3.40±0.65 to 1.22±0.42, P<0.001). Residual RV volume reduced to 71.96±3.8% of the expected volume and there was a significant negative correlation (rho −0.83) between TTA 'Z' score and indexed residual RV volume. During the follow-up of 20.54±7.62 months, the functional class improved from 2.59±0.7 to 1.34±0.52 (P<0.001). Conclusion: In Ebstein's anomaly, a higher TTA 'Z' score correlates with a lower postplication indexed residual RV volume. Hence, a complete trileaflet repair with offloading of RV by BCPS (when the TTA 'Z' score is >2) is recommended. The short-term outcomes of our technique are promising.

The experience of surgical treatment for Ebstein anomaly in 141 children / 中华外科杂志

Objective@#To explore the surgical strategy for Ebstein anomaly in children.@*Methods@#From January 2003 to December 2015, a total of 141 cases of Ebstein anomaly were treated at Department of Pediatric Cardiothoracic Surgery, Shanghai Children′s Medical Center, School of Medicine, Shanghai Jiaotong University. There were 65 male and 76 female patients, with age of (6.9±1.6) years (ranging from 10 months to 15 years), weight of (19.6±4.7) kg (ranging from 6.5 to 59.0 kg). All patients were diagnosed by 2 dimensional Doppler echocardiography and the septal leaflet and posterior leaflet displaced downward from 1.0 to 5.0 cm. The tricuspid valve regurgitation (TR) were mild in 26 cases, moderate in 46 cases and severe in 69 cases. Tricuspid valvuloplasty were performed in 131 cases (94 cone reconstruction, 37 valve hoist), tricuspid valve replacement in 2 cases and tricuspid valve closed in 8 cases. Surgical strategy were divided into biventricular heart function in 77 cases, one and a half ventricular heart function in 56 cases, and single ventricular heart function in 8 cases.@*Results@#Three patients were changed to one and a half ventricular repair from biventricular repair due to unstable hemodynamics in the early postoperative period. One case died in biventricular group. The complete atrioventricular block were occurred in 3 patients and pacemaker were applied. One hundred and forty cases discharged from hospital. There were mild TR in 118 cases, moderate in 14 cases and closed in 8 cases. One hundred and thirty-seven cases were followed up regularly in 18 to 172 months. Ninety-one cases were treated by cone reconstruction (mild TR in 75 cases, moderate in 15 cases and severe in 1 case). Thirty-six cases were operated by tricuspid valve hoist (mild TR in 21 cases, moderate in 12 cases and severe in 3 cases). In the patients with severe TR (4 cases), 3 cases were reoperated by cone reconstruction. One case′s valve was closed because of the dysplasia of the anterior valve and then from one and a half ventricular heart function to single ventricular function, the oxygen saturation was increased. Two patients underwent tricuspid valve replacement, 1 died and the other′s mechanical valve was removed, and changed to single ventricular function repair.@*Conclusions@#Although tricuspid cone reconstruction can achieve good results, the stable hemodynamic of early postoperative can be effectively maintained by using the surgical strategy of one and a half ventricular repair. To the patients with severe tricuspid regurgitation and hypoxemia due to severe tricuspid valve dysplasia, transforming to a functional single ventricle may be the only choice when there comes to the unstable hemodynamic.

The outcomes of operation for 237 patients with Ebstein anomaly / 中华外科杂志

Objective@#To evaluate the early and long-term outcomes cardiac surgery of patients with Ebstein anomaly.@*Methods@#The clinic data of 237 patients with Ebstein anomaly received surgical procedures from March 2004 to December 2017 at Department of Cardiac Surgery, First Hospital of Tsinghua University was analyzed retrospectively. There were 105 male and 132 female patients with age of (19.4±16.7) years (ranging from 3 months to 64 years). The surgical procedures include anatomical repair in 188 patients, one and a half ventricle repair in 37 patients, tricuspid valve repair in 4 patients, tricuspid valve replacement in 10 patients, and Fontan procedure in 3 patients (total cavopulmonary connection in 2 patients; Glenn procedure in 1 patient).@*Results@#The early mortality was 2.1% (n=5). One case of atrioventricular (0.4%) newly occurred. There were 228 patients available to follow-up. The range of follow-up duration was 3 to 168 months. Late survival was 99.1% (2 cases of late death) at 10 years. Three patients received reoperation (1.3%), including tricuspid valve repair of 1 patient and one and a half ventricle repair of 2 patients). Indication of tricuspid valve regurgitation improved from 3.6±0.3 to 1.5±0.4. Survival rate at 5 and 10 years was 98.6% and 98.2%, respectively.@*Conclusions@#The principle of the techniques is to reconstruct the tricuspid valve and right ventricle anatomically. For most cases, the anatomical repair was demonstrated with low mortality, less complications and excellent durability at long-term follow-up. If the tricuspid valve is severely hypoplastic, one and a half ventricle repair and valve replacement may be alternatie.