• Title/Summary/Keyword: Ebstein's anomaly

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Ebstein`S Anomaly: A Case Report of Plication and Tricuspid Valve Replacement (Ebstein 심기형 수술 1례[Plication 및 삼첨판막 이식예])

  • 송명근
    • Journal of Chest Surgery
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    • v.11 no.3
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    • pp.342-347
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    • 1978
  • A 8 year old male was admitted to the Department of Thoracic Surgery, Korea University Hospital on June 22, 1978. The chief complaints were cyanosis and exertional dyspnea since at birth. EKG shows BVH and dextrocardia, phonocardiogram revealed the accentuation of second heart sound in aortic area. Echocardiogram from the left ventricle to the base of the heart, there is a discontinuity between the ventricular septum and the anterior aortic margin with a large aortic root & aortic overriding. His cardiac catheterization data and cardiac angiogram shows situs inversus totalis, dextrocardia, right aortic arch, large ventricular septal defect etc., and finally diagnosed Truncus Arteriosus. Edwards type IV with retrograde aortogram and selective bronchial angiogram. This is the first operative case reported as Rastelli operation for Truncus Arteriosus type IV in the literatures in Korea. Authors have experienced I case of Truncus Arteriosus, Edward type IV and Rastelli operation with Dacron Arterial Conduit Graft under cardiopulmonary bypass on July 3, 1978. The procedures were as follows; 2] Cardiopulmonary bypass: Origin of bronchial arteries excised from descending aorta bilaterally; defects in aorta closed. 2] Horizontal incision made high in right ventricle. 2] Ventricular septal defect [Kirklin type I+II] closed with Teflon patch. 4] Bifurcated dacron arterial graft with pericardial monocusp sutured to the bilateral pulmonary arteries. [Diameter 9 mm: Length 7 cm]. 5] Proximal end of the conduit graft anastomosed to right ventricle. [Diameter 19 mm: Length 5 cm]..Total perfusion time was 220 min. The result of operation was poor due to anastomotic leakage and increased pulmonary vascular resistance resulting acute right heart failure. The patient was died on the operation table. Literatures were briefly reviewed.

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Open Heart Correction Of Ebstein`S Anomaly: A Report Of 8 Cases (Ebstein`s 심기형의 개심수술 8예)

  • 김삼현
    • Journal of Chest Surgery
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    • v.14 no.4
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    • pp.388-398
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    • 1981
  • Fourteen Infants with congenital cardiac anomalies underwent primary surgical Intervention within the first 12 months of life. There were eight patients with ventricular septal defect, two with total anomalous pulmonary venous return [TAPVR], and the remainders with tetralogy of Fallot, transposition of great arteries [d-TGA], Taussing-Bing malformation, and coronary A-V fistula. The age of the patients ranged from 5 to 12 months, with a mean age of 9.9 months. The mean weight was 6.7 Kg [3.8 to 9.5 KS]. Congestive heart failure persisting despite intensive medical treatment was present In 8 patients [56%], and was the most common indication for operation. Early operation was necessary in 5 of these patients [35%], because of failure to thrive and recurrent pulmonary infection. In one patient with TOF, frequent hypoxic spell prompted the necessity for early operation. In cases of VSD, TAP. VR, TOF, and coronary A-V fistula, Intracardiac repair was done with conventional cardiopulmonary bypass, chemical cold cardioplegia, and topical myocardial cooling. Deep hypothermic circulatory arrest with surface induced cooling, followed by core cooling and core rewarming, was employed .for better exposure in the cases of d-TGA and Taussing-Bing malformation. The results were however, not satisfactory. The overall mortality was 28 per cent. There were no deaths in the eight patients with VSD. The one with coronary A-V fistula survived. The other 5 cases all expired either on the table or immediately after operation. The non-fatal post-operative complications included low cardiac output, respiratory insufficiency, bleeding, and temporary A-V block. The causes of death were prolonged circulatory arrest time in d-TGA, complete A-V block and low cardiac output in TOF and Taussing-Bing malformation and prolonged bypass time and Inadequate correction in TAPVR.

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Clinical Analysis of 622 Cases of Open Heart Surgery (1986 년 개심술 622례에 대한 임상적 고찰)

  • 박표원
    • Journal of Chest Surgery
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    • v.20 no.3
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    • pp.489-497
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    • 1987
  • Six hundred and twenty two cases of open heart surgery were performed at Sejong General Hospital in 1986. And also, 117 cases of non open heart cardiac surgery had been performed during same period. Among the 622 open heart cases, 548 were congenital cardiac diseases and 74 were acquired heart diseases. In congenital heart patients, 422 were acyanotic and 126 were cyanotic. There were 52 cases of infant open heart Surgery below 12 months. Acyanotic group were consisted of 314 VSD, 66 ASD, 13 AVSD, 9 PDA, 8 ASD + PS, 4 AS, and 8 other rare cardiac cases. And cyanotic group were consisted of 84 TOF, 15 DORV, 5 Trilogy, 4 Ebstein`s anomaly, 3 PS + TR, 3 TGA, 3 TAPVR, 3 Pulmonary atresia and 6 other rare cardiac diseases. Majority of the acquired heart cases were valvular heart diseases. And there were also 4 cardiac myxoma and one endomyocardial fibrosis in acquired heart disease group. The operative results were as follows: Overall operative mortality, 5.3%: acyanotic 2.4%: cyanotic 15.8% and acquired heart disease, 8.5%.

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Early and Mid-term Results for Repair of Ebstein's Anomaly (엡스타인 기형에 대한 수술적 치료의 조기 및 중기 결과)

  • Lee Chang-Ha;Hwang Seong Wook;Lim Hong Gook;Kim Woong-Han;Kim Chong Whan;Lee Cheul
    • Journal of Chest Surgery
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    • v.38 no.4 s.249
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    • pp.284-290
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    • 2005
  • We evaluated the early and mid-term results for repair of Ebstein's anomaly. Material and Method: Between January 1989 and June 2004, 29 patients underwent repair of Ebstein's anomaly. The median age was 11.4 years (4 days-50 years). Tricuspid insufficiency equal to or greater than grade 3 was present in 21 $(72.4\%)$ patients. Surgical techniques included tricuspid valve repair with vertical plication of the atrialized ventricle (n=14), Carpentier's technique (n=7), tricuspid valve replacement (n=4), systemic-to-pulmonary arterial shunt (n=2), tricuspid valve repair (n=1), and Fontan operation (n=1). Bi-directional cavopulmonary shunt (BCPS) was required in 5 patients. Among the 2 neonates, one patient underwent successful biventricular repair, and the other patient underwent systemic-to-pulmonary arterial shunt. Follow-up was possible in 21 patients $(75\%)$, and the average follow-up was 37.6 months (3 months-11.3 years). Result: There were $1(3.4\%)$ early and 1 late deaths. Reoperation was required in 4 patients. Two patients underwent tricuspid valve re-replacement, and the other 2 tricuspid valve repair. At recent follow-up, only 2 patients showed tricuspid insufficiency equal to or greater than grade 3, and most patients showed clinical improvement. Excluding the patients who underwent tricuspid valve replacement, the actuarial rate of freedom from reoperation at 1 and 5 years were $94.7\%\;and\;79.0\%$, respectively. Conclusion: Tricuspid valve repair was possible in most patients with good mid-term outcome. Most patients showed clinical and hemodynamic improvement. Indications for the BCPS should be clarified.

Surgical Repair for Ebstein's Anomaly (Ebstein 기형의 수술 -2례 보고-)

  • naf
    • Journal of Chest Surgery
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    • v.12 no.3
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    • pp.289-296
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    • 1979
  • For years, physicians and anatomists have been interested in the heart that has one functioning ventricle. Various terms have been suggested for this entity including single ventricle, common ventricle, double-inlet left ventricle, cor biatriatum triloculare, and primitive ventricle. In this report, the term "single ventricle" is utilized as suggested by Van Praagh, and is defined as that congenital cardiac anomaly in which a common or separate atrioventricular valves open into a ventricular chamber from which both great arterial trunks emerge. An outlet chamber, or infundibulum, may or may not be present and give rise to the origin of either of the great arteries. This definition excludes the entity of mitral and tricuspid atresia. An 11 year old cyanotic boy was admitted chief complaints of exertional dyspnea and frequent upper respiratory infection since 2 weeks after birth. He was diagnosed as inoperable cyanotic congenital heart disease, and remained without any corrective treatment up to his age of 11 year when he suffered from aggravation of symptoms and signs of congestive heart failure for 2 months before this admission. On 22nd of May 1979, he was admitted for total corrective operation under the impression of tricuspid atresia suggested by a pediatrician. Physical check revealed deep cyanosis with finger and toe clubbing, and grade V systolic ejection murmur with single second heart sound was audible at the left 3rd intercostal space. Development was moderate in height [135 cm] and weight[28Kg]. Routine lab findings were normal except increased hemoglobin [21.1gm%], hematocrit [64 %], and left axis deviation with left ventricular hypertrophy on EKG. Cardiac catheterization and angiography revealed 1-transposition of aorta, pulmonic valvular stenosis, double inlet of a single ventricle with d-loop, and normal atriovisceral relationship [Type III C solitus according to the classification of Van Praagh]. At operation, longitudinal incision at the outflow tract of right ventricle in between the right coronary artery and its branch [LAD from RCA] revealed high far anterior aortic valve which had fibrous continuity with mitral annulus, and pulmonic valve was stenotic up to 4 mm in diameter positioned posterolaterally to the aorta. Ventricular septum was totally defective, and one markedly hypertrophied moderator band originated from crista supraventricularis was connected down to the imaginary septum of the ventricular cavity as a pseudoseptum of the ventricle. Size of the defect was 3X3 cm2 in total. Patch closure of the defect with a Teflon felt of 3.5 x 4 cm2 was done with interrupted multiple sutures after cut off of the moderator band, which was resutured to the artificial septum after reconstruction of the ventricular septum. Pulmonic valvotomy was done from 4 mm to 11 mm in diameter thru another pulmonary arteriotomy incision, and right ventriculotomy wound was closed reconstructing the right ventricular outflow tract with pericardial autograft of 3 x 4 cm2. Atrial septal defect of 2 cm in diameter was closed with 3-0 Erdeck suture, and atrial wall was sutured also when rectal temperature reached from 24`C to 35.5`C. Complete A-V block was managed with temporary external pacemaker with a pacing rate of 110/min. thru myocardial wire, and arterial blood pressure of 80/50 mmHg was maintained with Isuprel or Dopamine dripping under the CVP of 25-cm saline. Consciousness was recovered one hour after the operation when his blood pressure reached 100 /70 mmHg, but vital signs were not stable, and bleeding from the pericardial drainage and complete anuria were persisted until his heart could not capture the pacemaker impulse, and patient died of low output syndrome 320 min after the operation.

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Evaluation of Prognostic Factors in Corrected Transposition of the Great Arteries at Mid-term Follow-up (수정 대혈관 전위 환자에서 예후에 영향을 주는 인자들에 대한 중기적 고찰)

  • Song, Young-Hwan;Kwon, Hyok-Joo;Kim, Gi-Beom;Kang, Soo-Jung;Bae, Eun-Jung;Noh, Chung-Il;Yun, Yong-Soo;Lee, Jeong-Ryul;Kim, Yong-Jin;Rho, Joon-Ryang
    • Clinical and Experimental Pediatrics
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    • v.46 no.2
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    • pp.154-161
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    • 2003
  • Purpose : The prognosis of patients with corrected transposition of the great arteries(C-TGA) is variably affected by associated intracardiac defects, systemic right ventricular function, tricuspid valve competence, and conduction disturbances. This study aims to evaluate the importance of those factors at mid-term follow-up. Methods : Medical records of 94 patients(males 58, females 36; mean age at last follow-up, $12{\pm}9$ years; mean follow-up duration, $9{\pm}6.4$ years) diagnosed between January 1980 and May 2002 at Seoul National University Children's Hospital were studied retrospectively. Results : Among 94 patients, operations were performed in 72 patients(classic operations in 55; double switch operations in 17). Among prognostic factors including associated intracardiac anomalies(at least moderately severe tricuspid insufficiency(TI), ventricular septal defect, pulmonary stenosis and pulmonary atresia), intracardiac operation and complete atrioventricular block, TI was the only significant factor for death(P=0.001), and in turn, Ebstein anomaly and high grade atrioventricular block predicted TI. 20-year survival without TI was 77%, but only 35% with TI(P=0.0002); excluding perioperative death, the 20-year survival rates with and without TI were 48% and 87% respectively(P=0.008). There was no statistical difference in 20-year survival rate or association with TI between classic and double switch operation. Conclusion : TI was the major prognostic factor for C-TGA and was associated with Ebstein anomaly and high grade atrioventricular block at mid-term follow-up. Long-term follow-up is required to evaluate other factors, including double switch operations and associated intracardiac defects more exactly.

Open Heart Surgery of Infants Uunder 10kg of Body Weight (체중 10kg 이하 영아에서의 개심술)

  • 박성동
    • Journal of Chest Surgery
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    • v.28 no.5
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    • pp.447-452
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    • 1995
  • From January 1983 to December 1992, 116 patients under 10Kg of body weight were underwent open heart surgery in National Medical Center.There were 64 males and 52 females, and their mean age was 14 2.6 months, ranged from 3 days to 38 months. Their mean body weight was 7.4$\pm$1.2Kg, ranged from 2.4Kg to 10Kg which were less than 3 percentile of normal developmental pattern in 109 out of 116 cases.The composition of the patients was VSD[73 , TOF[18 , ASD[8 , ECD[5 , TGA[4 , PS[3 , TAPVC[2 , TA[1 , cardiac rhabdomyoma with ASD[1 , and Ebstein`s anomaly[1 . The overall surgical mortality was 17.2%.In acynotic group, 11 patients died among 91 patients and the mortality was 12.1%, in cyanotic group 9 patients died among 25 patients, the mortality was 36%.The surgical result of patients under 5Kg of body weight was worse than those over 5Kg of body weight. [56.2% versus 11% The surgical result in this period was improved year by year, but poor surgical results in the group of cyanotic, under 5Kg of body weight should be improved by means of more precise preoperative diagnoses, more advanced surgical techniques and more proper postoperative management.

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Clinic Analysis of Heart Surgery -110 cases- (심장수술 110례 임상고찰)

  • Chang, Hoon;Shin, Sung-Aia;No, Joong-Kee;Rho, Jun-Ryang
    • Journal of Chest Surgery
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    • v.34 no.8
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    • pp.597-603
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    • 2001
  • Background: The purpose of writing this article is to get better clinical results and further clinical improvement based on subject to 110 cases of cardiac surgery which were performed and clinically analyzed. Material and Method: Since January 1995, the patent ductus arteriosus surgery had started in our hospital. In February 1999, an open heart surgery had started and up to September 2000, total of 110 cases were performed as of double ligation of patent ductus arteriosus(10 cases) and open heart surgery(100 cases). Result: Among the patients, Korean-Chines was 74(67.3%) and Han-Chinese was 35(31.8%). Congenital heart disease was 95 cases and acquired valvular heart disease was 15 cases. 83 cases of acyanotic congenital heart disease consisted of ventricular septal defect(VSD) with associated anomaly(45 cases), atrial septal defect(ASD) with associated anomaly(20 cases), patent ductus arteriosus(PDA) with associated anomaly(11 cases), cogenital aortic stenosis(5 cases), double chamber right ventrical(1 case) and Ebstein's anomaly(1 case). Among the 12 cases of cyanotic congenital heart disease, 11 cases of tetralogy of Fallot underwent total correction. Among the 15 cases of acquired valvular heart disease, valvular replacement(7 cases), double valve replacement (3 cases), mitral valve replacement(3 cases) and aortic valve replacement(1 case) were performed. And 8 cases of valvuloplasty were performed by using of commissurotomy, chordal plasty, plasty of papillary muscle, ring type annuloplasty, repair of leaflet. Conclusion: on cogenital heart disease, short term results of surgery for acyanotic cogenital heart disease was good. Among the cyanotic congenital heart disease, tetralogy of Fallot showed a little difference of recovery according to the surgery method so that further follow up observation was needed for long term result. On acquired valvular heart disease, especially, in terms of short term result of valvuloplasty, was relatively good, but further follow up observation was also needed for long term result. There wasn's any operative mortality.

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Clinical Experience of Open Heart Surgery; 1000 Cases (심장수술 1,000례의 임상적 고찰)

  • 조광현
    • Journal of Chest Surgery
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    • v.26 no.4
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    • pp.282-293
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    • 1993
  • From Sep. 1985 to Dec. 1992, total 1000 cases of open heart surgery [OHS] were performed in the department of Thoracic & Cardiovascular Surgery, Pusan Paik Hospital, College Of Medicine, Inje University.Among the total 1,000 cases of OHS, there were 823 cases with congenital heart diseases [CHD] and 177 cases with acquired heart diseases [AHD]. The age distribution was 9 days [4.0 kg] to 49 years in CHD and 11 to 64 years in AHD, In the 823 cases of CHD, there were 763 acyanotic cases and 60 cyanotic cases. The CHD cases consisted of 520 VSD [63.2 %], 177 ASD [21.5 %], 60 TOF[7.3 %], 27 PS [3.3 %], 17 ECD [2.1%], 7 Valsalva sinus rupture [0.9 %], 4 TGA [0.5 %], 3 Ebstein`s anomaly [0.4%], 3 DORV[0.4%], and others. The corrective operations were applied for congenital heart disease with the result of 2. 8 % hospital mortality. In the 177 AHD, 168 cases were valvular heart diseases, 7 cases were cardiac tumors and one LA thrombus and one annuloaortic ectasia. In the 168 valvular heart diseases, there were 115 single valve replacement cases [16 AVR, 99 MVR], 20 cases of double valve replacement[AVR & MVR], 15 cases of MVR with TVA, and 10 cases of AVR, MVR with TVA. The total implanted prosthetic valves were 199`. In MVR, 66 of St. Jude Medical valves, 78 ofCarpentier-Edward valves, and 5 of Ionescu-Shiley valves were used. In AVR, 38 of St. Jude Medical valves and 12 of Carpentier-Edward valves were used.The hospital operation mortality rate of congenital acyanotic, cyanotic, and acquired heart diseases were 1.6%, 18.3 % and 3.4% respectively. The overall mortality rate was 2.9 % [29/1000].

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Long-Term Result of Tricuspid Valve Replacement (삼첨판막 치환술의 장기성적)

  • Lim, Cheong;Kang, Moon-Chul;Kim, Kyung-Hwan;Kim, Ki-Bong;Ahn, Hyuk
    • Journal of Chest Surgery
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    • v.34 no.9
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    • pp.680-685
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    • 2001
  • Background: Tricuspid valve replacement is very rarely performed procedure and its long-term result is not yet satisfactory. Moreover, it is not well known whether bioprosthesis or mechanical prosthesis is the best selection for artificial valve. We reviewed 72 cases of tricuspid valve replacements in 71 patients between January 1989 and December 1998, trying to analyze the overall results and risk factors for mortality and morbidity. Material and Method: Average age of the patients at the time of operation was 42$\pm$13 years(range 16 to 65 years) and the sex ratio of male versus female was 32/39. Primary diagnosis consisted of 50 cases of aquired valvular heart disease and 18 cases of congenital heart disease, such as Ebstein’s anomaly. 4 cases had isolated tricuspid valve regurgitation. Implanted valves were 69 mechanical prosthesis and 3 bioprosthesis. Concomitant mitral or aortic valve replacements were performed in 50 cases. One patient received concomittant pulmonary valve replacement. Result: There were 7(9.72%) operative deaths and 7(13.0%) late deaths. Actuarial survival at 10 years was 59.2$\pm$7.2%. Prosthetic tricuspid valve thrombosis occurred 11 times in 5 patients. Reoperation for prosthetic tricuspid valve failure was performed in 1 patient. In this case, examination of the explanted prostheses showed that the tricuspid stenosis was the result of valve thrombosis. Among the 47 survivors, 46 patients(98%) were in functional class I or II. Conclusion: In our ten-year experience of tricuspid valve replacement, mortality and morbidity were satisfactory. Mechanical prosthesis in tricuspid position showed comparable clinical results as bioprosthesis.

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