• Korean Journal of Clinical Laboratory Science
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• v.51 no.2
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• pp.265-269
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• 2019

Ebstein's anomaly is attached to the right ventricle with the tricuspid leaf attached to the lower right ventricle without any attachment to the tricuspid annulus. Most patients in their 20s are alive. Patients older than 25 years of age are similar in the incidence of heart failure and sudden death, and survive approximately 70% from 2 years, and 50% from 3 years, from 5% to over 50 years of age. Through this example, Ebstein's anomaly exists in various forms. A 22 year old case with WPW syndrome and those who survived to 77 years of age are reported. The normal heart structure in the apical four-chamber view should be observed on similar lines, even though the tricuspid annulus is slightly lower than the mitral annulus. If not, there will be some doubt regarding this anomaly. Furthermore, echocardiography plays an important role in the diagnosis and prognosis of congenital heart disease.

• Journal of Chest Surgery
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• v.16 no.2
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• pp.157-163
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• 1983

Ebstein's anomaly is a rare congenital cardiac malformation oand the ideal surgical correction seems controversial at present, and some problems are left unsolved in the surgical correction of this anomaly. Between June 1978 and June 1982, 12 patients with Ebsteins' anomaly underwent corrective open heart surgery at Seoul National University Hospital. Except for one patient, who had no ASD, all had a huge right atrium, secundum type ASD, and definite atrialized right ventricle. Typically, displaced tricuspid valve leaflets were found in all cases, but the degree of displacement and deformity were variable. In the point of NYHA functional classification, five were in class II, six were in class III, and one was in class IV. Ten patients were operated on by tricuspid valve replacement and pication. Two patients were operated on only by plication and annuloplasty techniques, and in all cases, ASD was closed. Postoperatively, four patients suffered from complete A-V block, and two of them died immediately. The remaining two patients took pacemaker generator implantation with good results. The other eight patients were in good condition. Tricuspid valve replacement using tissue valve and plication of the atrialized rght ventricle seems to be a good method of surgical correction for Ebstein's anomaly.

• Journal of Chest Surgery
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• v.35 no.4
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• pp.303-306
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• 2002

Patients with severe Ebstein's anomaly showing in the neonatal period, represent progressive cardiac enlargement with pulmonary hypoplasia and functional pulmonary atresia with patent ductus alteriosus-dependent pulmonary circulation. Biventricular repair in these patients had been mostly unsuccessful except for Starnes' procedure that converts the anatomy to single ventricle physiology for Fontan procedure. A 4-days old male was admitted with the diagnosis of severe Ebstein's anomaly with anatomic pulmonary atresia and severe cardiac enlargement. He successfully underwent biventricular repair with vertical plication method of atrialized right ventricle, tricupid annuloplasty, transannular right ventricular outflow tract reconstrulltion, atrial septal defect patch closure with fenestration, and right atrial reduction angioplasty Postoperatively, cardiothoracic ratio was significantly reduced and mild tricuspid regurgitation was remnant in echocardiography. The patient is currently 10 months old and is fully active without restrictions.

• Journal of Chest Surgery
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• v.31 no.2
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• pp.216-219
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• 1998

Ebstein's anomaly is a complex malformation that can be treated by various surgical techniques, either repair or replacement of the abnormal tricuspid valve, with variable results. The essence of the malformation is the downward displacement of the septal and posterior leaflets into the ventricle, resulting in the formation of an atrialized portion of the right ventricle. The aim of surgical repair is to correct the tricuspid valve dysfunction and to plicate the atrialized portion of the right ventricle A 12-months old female was admitted with the diagnosis of Carpentier type A of Ebstein's anomaly with severe tricuspid regurgitation. She successfully underwent operation with vertical plication of right ventricle and reimplantation of tricuspid leaflets. Postoperatively cardiac size was significantly reduced and tricuspid regurgitation was trivial in echocardiography. She was diacharged the 14th postoperative day.

• Journal of Chest Surgery
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• v.40 no.7 s.276
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• pp.473-479
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• 2007

Background: The Hetzer procedure for the correction of Ebstein's anomaly has the advantages of technical feasibility and incorporation of the atrialized right ventricle (RV) into the functional RV. Material and Method: We preformed a retrospective review of 11 patients with Ebstein's anomaly and they had undergone a Hetzer procedure between March 2002 and December 2006. Result: The median age at operation was 19.8 years (range: 6 months ${\sim}56$ years). There were 4 males and 7 females. All patients showed severe tricuspid regurgitation (TR) preoperatively, and arterial desaturation (<95%) was present in 3 patients. The original Hetzer technique was employed in 6 patients with the Carpentier type A anomaly. In the remaining 5 patients with the Carpentier type B or C anomalies, valve competence was restored at the level of the displaced tricuspid valve mechanism. Adjunct bidirectional cavo-pulmonary shunt, or one and a half ventricle repair strategy was employed for all the patients. The median follow-up was 8.6 months (range: $0.8{\sim}51.9$ months). There was no early or late death, and the immediate postoperative TR was trivial to mild in 8 patients. The median cardio-thoracic ratios on chest X-ray at the preoperative period and at postoperative 0, 1 and 6 months were 65%, 62%, 55% and 55%, respectively. Conclusion: The original or modified Hetzer procedure for Ebstein's anomaly shows excellent intermediate-term outcomes.

• Journal of Chest Surgery
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• v.29 no.4
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• pp.381-385
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• 1996

From April 1984 to April 1990, seven patients with Ebstein's anomaly underwent surgical repair. Mean age at operation was 18.6 years (range, 2 to 46 years). Operations were performed using hypothermic cardiopulmonary bypass. Surgical procedures included tricupid valve replacement (n:6) and tricuspid valve reconstruction (n: 1). There were two hospital deaths. There have been no late death. All survivors are in New York Heart Association class I or II with a median follow-up of 6.2 years(range, 4 to 8.3 years).

• Journal of Chest Surgery
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• v.27 no.12
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• pp.1027-1030
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• 1994

We reviewed 10 cases of traumatic diaphragmatic injuries at Soonchunhyang University Gumi Hospital from January 1990 through April 1993. Seven patients were male and three patients were female. The age distribution was ranged from 25 to 79 years, predominant 4th decades occurred in male. The traumatic diaphragmatic injuries were due to blunt trauma in 9 cases[traffic accident 7 and crash injury 2] and penetrating wound in 1 case[stab wound]. The common symptoms were dyspnea[60%], chest pain and abdominal pain in order frequency. In the blunt trauma and crash injury, the rupture site was all located in the left[ 9 cases ]. In the penetrating wound, the rupture site was located in the right[1 case]. The surgical repair of 10 cases were performed with transthoracic approach in 9 cases and thoracoabdominal approach in 1 case. The postoperative mortality was 10%[1/10]. The cause of death was multiple organ failure with pulmonary edema.

• Clinical and Experimental Pediatrics
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• v.48 no.11
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• pp.1212-1218
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• 2005

Purpose : Forward pulmonary blood flow may be absent in some neonates with Ebstein's anomaly by anatomical or functional pulmonary atresia in association with the elevated pulmonary vascular resistance, patent ductus arteriosus and tricuspid regurgitation. We reviewed the presentation and outcomes of symptomatic neonates with Ebstein's anomaly focusing on the pulmonary atresia. Methods : Clinical presentation and outcome of 15 symptomatic neonates with Ebstein's anomaly seen at Asan medical center from 1998 to 2004 were reviewed. Results : Ten(67%) of 15 patients showed no forward pulmonary blood flow and 6 of them had functional pulmonary atresia. $O_2$ saturation and pH were lower and cardiothoracic(CT) ratio in chest radiography was more increased in the patients with pulmonary atresia than in the patients without pulmonary atresia(P<0.05). pH and CT ratio were not different between the anatomical and functional pulmonary atresia group, but $O_2$ saturation was lower in functional atresia group(P<0.05). 13 patients(87%) were managed with $PGE_1$. 4 of 6 patients with functional pulmonary atresia were treated with inhaled nitric oxide. Surgery was performed in 1 of 5 patients without pulmonary atresia and in 8 of 10 patients with pulmonary atresia during follow-up period(mean 37 months). 3 patients(20%) died and none of patients without pulmonary atresia died. Conclusion : We found that most symptomatic neonates with Ebstein's anomaly had functional or anatomical pulmonary atresia. The neonates with Ebstein's anomaly who had no forward pulmonary blood flow were more symptomatic and needed surgery earlier. Further studies will be needed to distinguish effectively functional and anatomical pulmonary atresia and to manage appropriately neonates with functional atresia.

• Proceedings of the KTCVS Conference
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• 2004.06a
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• pp.164-167
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• 2004

• Journal of Chest Surgery
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• v.35 no.12
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• pp.890-893
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• 2002

Ebsteins anomaly is a rare congenital cardiac malformation that is the downward displacement of the septal and posterior leaflets of the tricuspid valve into the ventricular apex, aresulting in the formation of an atrialized portion of the right ventricle. In most patients, symptoms such as cyanosis and progressive heart failure occur during the adolescence or young adulthood. The associated anomalies include ventricular septal defect, transposition of the great arteries, and malformation of the mitral valve. An 8-months-old male with Ebsteins anomaly, ventricular septal defect, and duplication of the mitral valve orifice successfully underwent operation with VSD patch closure. Postoperatively, cardiac size was significantly reduced and tricuspid regurgitation was trivial in echocardiography He has been doing well without any cardiac medication during the 18 months follow up after operation.