• Journal of Chest Surgery
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• 제35권8호
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• pp.590-593
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• 2002

온전한 심실중격을 가진 폐동맥판막무형성증(absent pulmonary valve syndrome with intact ventricular septum, APVS with IVS)은 매우 드문 선천성 심장 질환이다. 신생아의 경우 확장된 폐동맥에 의한 기도폐쇄와 이로 인한 심한 호흡곤란증으로 높은 사망률을 유발한다. 이에 대한 해결책으로 여러 약물치료 및 여러 고식적 수술, 폐동맥 판막 삽입, 폐동맥 축소술(reductive angioplasty of pulmonary artery) 등이 사용되었다. 본원에서는 동맥관 결찰술, 폐동맥 축소술, 폐동맥 판막 재건술(pulmonary valve reconstruction) 및 심방중격결손 봉합술을 시행하여 좋은 결과를 얻었다.

• Clinical and Experimental Pediatrics
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• 제49권6호
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• pp.635-642
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• 2006

목 적 : 폐동맥판 역류를 감소시키고자 2000년 4월부터 자기 판막을 이용한 판막 재건술을 시행하였으며, 이러한 수술 방법이 폐동맥판 역류와 임상적인 결과의 호전을 주는지 알아보고자 본 연구를 시행하였다. 방 법 : 1991년 7월부터 2004년 8월까지 동아대학교 의료원에서 활로사징으로 완전 교정술을 시행한 환아 중, 자기 판막 조직을 이용한 폐동맥 판막 재건술을 시행한 31례(PVR군)와 수술 후 같은 추적 관찰 기간을 가진 활로사징 환아 중 경판륜 첩포(transannular patch) 확장 수술 후 단엽(monocusp)을 삽입한 47례(MVOP군), 경판륜 첩포 수술만 시행한 22례(TAP군)를 대조군으로 하였다. 이들 대조군과 PVR군에서 폐동맥판 역류, 폐동맥 판막의 기능, 삼첨판/승모판 판륜의 비율, 심-흉곽 비율을 계측하여 비교하였다. 결 과 : MVOP군에서 심초음파 검사상 중등도의 폐동맥판 역류는 수술 직후 35례(74%)에서 관찰되었고, 우심실의 확장이 있는 경우가 12례(26%)였다. 그러나 PVR군은 중등도의 폐동맥판 역류는 6례(19%)였고, 우심실의 확장이 있는 경우는 1례(3%)에 불과하였다. 이후 매년 추적 검사상 MVOP군에서 거의 모든 예에서 중등도의 폐동맥판 역류를 보였고, PVR군에서 점차 증가하는 경향을 보였다. MVOP군의 수술 직후 초음파 검사에서 운동성이 확인되지 않은 경우가 18례(38%)였으며, 12례에서는 양호한 판막 기능을 보였다. PVR군은 30례(96%)에서 우수한 판막 기능을 보여 주었고, 1례(3%)에서 양호한 판막 기능을 보였다. 추적 관찰 기간 중의 판막의 기능은 MVOP군에서 추적 관찰 기간에 따라 판막 기능의 급격한 감소를 보여 주었으며, PVR군은 1년, 2년, 3년, 4년 추적 관찰 기간에 각각 27례(90%), 21례(75%), 14례(68%), 8례(50%)로 관찰 기간이 경과할수록 기능이 감소하는 경향을 보였다. 심-흉곽의 비율은 세 군에서 수술 직후에는 차이가 없었으나 PVR군이 1년, 2년, 3년, 4년 추적 관찰 기간에 통계학적으로 유의하게 양군에 비해 감소되었다. 그러나 MVOP군과 TAP군간에는 통계학적으로 유의한 차이는 없었다. 삼첨판/승모판 판륜 직경의 비는 수술 직후에는 차이가 없었으며 추적 관찰 기간 1년, 2년 및 3년에 양 군에 비해 통계학적으로 유의하게 차이가 있었다. 그러나 MVOP군과 TAP군간에는 통계학적으로 유의한 차이는 없었다. 결 론 : PVR군이 다른 군에 비해 폐동맥판 역류의 정도는 감소되었으며, 판막의 기능이 양호하였고, 심-흉곽 비율, 삼첨판/승모판 판륜의 비가 유의하게 낮았다. 그러나 PVR 군에서 폐동맥판 역류 정도, 판막 운동의 저하가 추적 관찰 기간이 경과함에 따라 악화하는 경향을 보였으며, 장기적인 추적 관찰이 필요하리라 생각된다.

• Journal of Chest Surgery
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• 제32권3호
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• pp.303-306
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• 1999

본 증례는 Ross 술식에서 동종판막이나 이종판막을 쓰지않고 자가 대동맥 조직과 심낭으로 우심실 유출로를 성공적으로 재건한 보고이다. 선천성 대동맥판막 협착증을 진단 받은 8세 환아에서 시행한 폐동맥 자가 이식편을 이용하여 대동맥판을 교체하고 자가 대동맥 조직과 심낭편으로 단엽 판막을 만들어 우심실 유출로를 재건하였다. 술후 검사에서 심실과 새로운 대동맥판의 기능이 좋아 투약없이 19개월째 외래 추적관찰 중이다.

• Journal of Chest Surgery
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• 제22권2호
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• pp.348-356
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• 1989

Seventeen patients of pulmonary atresia with intact ventricular septum were underwent operation during 4.8years period from Jan. 1983 to Aug. 1988 at Seoul National university Hospital. The patients were composed of 8 males and 9 females, aging 1day to 2.5 years [mean 88 days]. We classified pulmonary atresia according to right ventricular morphology; those with tripartite ventricles in 12, those with no trabecular portion to the cavity in 0, and those with inlet portion only in 5. The tripartite approach to right ventricular morphology is helpful in selecting the type of initial palliative procedures. Palliative procedures were as follows; pulmonary valvotomy in 5 with 3 early survivors, mod B-T shunt in 4 with 3 early survivors, and palliative right ventricular outflow tract reconstruction in 4 with 1 early survivor. Effective preliminary palliation of pulmonary atresia are pulmonary valvotomy or palliative right ventricular outflow tract reconstruction in those with tripartite right ventricle, and modified Blalock-Taussig shunt in those with no infundibular portion. The approach to definitive repair is based primarily on the actual size of the tricuspid annulus and the right ventricular cavity. Definitive repair was as follows: definitive right ventricular outflow tract reconstruction in 4 with all survivors and mod. Fontan operation in 2 with one survivors. Right ventricular outflow tract reconstruction can be done as complete repair for patients who had adequate tricuspid annulus and right ventricular cavitary size and mod. Fontan operation for patients who severely hypoplastic tricuspid valve annulus or small right ventricular cavity.

• Journal of Chest Surgery
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• 제17권4호
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• pp.636-643
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• 1984

For the purpose of avoiding postoperative massive pulmonary insufficiency after transannular outflow tract reconstruction in patients with tetralogy of Fallot, we have used monocusp bearing outflow patch since June 1983. Right heart catheterization and pulmonary arteriography were performed in 7 patients among the total 11 patients corrected with monocusp bearing outflow patch during postoperative 14th day to 22nd day. Particular attention was paid to the evaluation of the pulmonary valve competence, and the results were; 1.One patient died of acute renal failure secondary to low cardiac output and the operative mortality was 9.1%. 2.The average PRV/FA ratio was 0.491 and the average systolic pressure gradient between right ventricle and pulmonary artery was 17.7mmHg. The average Qp/Qs was 1.13. 3.Inspite of using monocusp bearing outflow patch, the hemodynamic and pulmonary arteriographic results were unsatisfactory in respect to pulmonary valve competence.

• Journal of Chest Surgery
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• 제33권6호
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• pp.476-486
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• 2000

Background: Pulmonary atresia with intact ventricular septum(PA/IVS) is an anatomically heterogenous anomaly with a variety of surgical strategies possible. The purpose of the study is to evaluate the influence of right ventricular size on the early and midterm results of surgical repair of PA/IVS. Material and method: Medical records of 20 consecutive patients with PA/IVS operated on between January 1993 and August 1999 were retrospectively reviewed. There were 12 boys and 8 girls whose ages ranged from 2 days to 14.5 months (median 6 days). Their body weight ranged from 2.52kg to 9.35 kg(median 3.18kg). The preoperative Z-value of the diameter of the tricuspid valve(T-valve) was less than or -4 in 5 patients, between -4 and -2 in 1, between -2 and 0 in 7, between 0 and 2 in 6, and greater than or 2 in 1. All patients who had z-value of tricuspid valve greater than -2.05 were attempted biventricular repair(n=15) and all patients who had it smaller than -4.4 underwent systemic-pulmonary shunt operation only(n=3) or bidirectinal cavopulmonary shunt with right ventricular reconstruction(n=2). Result: Two early deaths(2/20, 10%) occurred. Both were infants who underwent transannular patch with shunt. One of these two had huge right ventricle(Z-value of tricuspid valve = 5). There were 2 late non-cardiac deaths 3 and 7 months after operations respectively. Follow-up was completed in all children at a mean of 35.3 months(range, 5 to 54 months). 10 of 11 survivors who underwent transannular patch or valvotomy with or without shunt procedure were in NYHA functional class I even though some of them had small interatrial communication or patent shunt. All three patients who had shunt procedure only at initial palliation completed Fontan procedures with no death. Two patients who underwent right ventricular outflow reconstruction with bidirectional cavopulmonary shunt were also in good condition. Conclusion: The transanular RVOT patch or valvotomy with or without systemic-pumonary shunt as an initial palliative procedure to achieve biventricular repair for the patients who had neither too small nor too large right ventricle(-2.05$\leq$Z-value of T-value of T-valve$\leq$2) could be performed at low operative risk(1/14 7.1%). Systemic-pulmonary shunt procedure and bidirectional cavopulmonary shunt procedure for the patients who had small right ventricle(Z-value of T-valve$\leq$4.4) could be also performed with low risk. But a patient with huge right atrium and ventricle(Z-value of t-valve=5) had poor operative result.

• Journal of Chest Surgery
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• 제16권3호
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• pp.337-341
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• 1983

The experience with operative treatment for total correction of Tetralogy of Fallot at the department of Thoracic and Cardiovascular Surgery, Korea University Hospital from January, 1977, through April, 1983 was reviewed. Of the 29 patients reviewed, male to female occurrence ratio was 22:7 Type of V.S.D. was type II in 26 cases[90%] and total conus defect in 3 cases[10%]. Average size of V.S.D. was 19 mm. Type of Right ventricular outflow tract stenosis was highest frequency with pulmonary valvular and infundibular stenosis combined type in 21 cases[72%], and there were 8 deaths In this group. Type of R.V.O.T. reconstruction contains 2 cases of infundibulectomy only, 1 case of infundibulectomy with valvular commissurotomy, 2 cases of pericardial patch, 23 Cases of pericardial patch with Teflon or Dacron felt reinforced and 1 case of pulmonary valved conduit reconstruction. Operative mortality was higher in outflow patch through pulmonary valve ring. Overall mortality was 31%. Major causes of death and postoperative complications were low output syndrome, complete A-V block acute renal failure, ventricular fibrillation, bleeding brain abscess, and sudden cardiac arrest.

• Journal of Chest Surgery
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• 제33권10호
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• pp.830-833
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• 2000

Homograft has been the conduit of choice in various types of congenital malformations which require right ventricular outflow tract reconstruction. However it has been proven to be less than ideal in young age group because of early failure of the conduite due to valve dysfunction and calcification. Furthermore limitation of availability of homograft particularly small sized conduits for neonates and infants is the most serious problem. A 19 month old female patient with pulmonary atresia and ventricular septal defect was operated on with a bovine jugular venous valved conduit as an alternative to the homograft for her right ventricular outflow tract reconstruction. Postoperative hemodynamic performance of the conduit was excellent without pressure gradient or valve regurgitation. With this early result bovine jugular venous valved conduit seems to be another excellent conduit because of good hemodynamics and size availability but long term follow up is necessary.

• Journal of Chest Surgery
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• 제49권5호
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• pp.344-349
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• 2016

Background: Monocusp reconstruction with a transannular patch (TAP) results in early improvement because it relieves residual volume hypertension during the immediate postoperative period. However, few reports have assessed the long-term surgical outcomes of this procedure. The purpose of the present study was to evaluate the mid-term surgical outcomes of tetralogy of Fallot (TOF) repair using monocusp reconstruction with a TAP. Methods: Between March 2000 and March 2009, 36 patients with a TOF received a TAP. A TAP with monocusp reconstruction (group I) was used in 25 patients and a TAP without monocusp reconstruction (group II) was used in 11 patients. We evaluated hemodynamic parameters using echocardiography during the follow-up period in both groups. Results: At the most recent follow-up echocardiography (mean follow-up, 8.2 years), the mean pulmonary valve velocities of the patients in group I and group II were $2.1{\pm}1.0m/sec$ and $0.9{\pm}0.9m/sec$, respectively (p=0.001). Although the incidence of grade 3-4 pulmonary regurgitation (PR) was not significantly different between the two groups (group I: 16 patients, 64.0%; group II: 7 patients, 70.0%; p=0.735) during the follow-up period, the interval between the treatment and the incidence of PR aggravation was longer in group I than in group II (group I: $6.5{\pm}3.4years$; group II: $3.8{\pm}2.2years$; p=0.037). Conclusion: Monocusp reconstruction with a TAP prolonged the interval between the initial treatment and grade 3-4 PR aggravation. Patients who received a TAP with monocusp reconstruction to repair TOF were not to progress to pulmonary stenosis during the follow-up period as those who received a TAP without monocusp reconstruction.

• Journal of Chest Surgery
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• 제29권8호
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• pp.822-827
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• 1996

소아에서 대동맥 판막 질환의 경우 합리적인 수술법인 Ross술식을 적용함에 있어,우리 나라의 경우 현실적으로 이용에 어려움이 있는 Ross술식에 필요한 동종이식 판막의 사용을 배제한다는 발상으로 10마리의 15 kg가량의 돼지를 이용 REV술식 등을 시행하고 3마리의 성공 례를 3개월 이상 사육하여 성돈이 된 후 그 판막의 발육 및 기능을 관찰하였다. 대동맥 및 폐동맥 등에 협착의 소견은 없었으며, REV술식으로 사용되었던 monocusp보철편의 변형 및 심한 석회화가 관찰되어 이미 판막으로의 기능 은 상실하였으나 발육에는 큰 지장이 없는 것이 확인되어 REV와 Ross술식의 병행 수술요법이 사람에 서도 적 용될 수 있을 것으로 사료되 며, 계속 석회화 방지에 관한 연구가 진행되면 더욱 좋은 성적을 기대할 수 있겠다.