• Journal of Chest Surgery
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• v.35 no.8
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• pp.590-593
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• 2002

Absent pulmonary valve syndrome with intact ventricular septum(APVS with IVS) is a rare congenital anomaly. The severe form of this syndrome, characterized by severe respiratory distress presented soon after birth, has been attributed to the compression of the airways caused by aneurysmal dilatation of pulmonary artery. Several operative treatments such as pulmonary valve insertion, or reductive angioplasty of pulmonary artery have been applied. We present a 3-day-old male who showed improvement after PDA ligation, reductive angioplasty of pulmonary artery, pulmonary valve reconstruction, and ASD closure.

• Clinical and Experimental Pediatrics
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• v.49 no.6
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• pp.635-642
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• 2006

Purpose : The purpose of this study is to determine whether the new pulmonary valve reconstruction technique prevents short-term postoperative pulmonary regurgitation and improves early and mid-term clinical outcome. Methods : We reviewed postoperative echocardiographic variables and chest X-ray films from 31 patients who had undergone valve reconstruction(pulmonary valve reconstruction group : PVR) for the repair of TOF between April 2000 and August 2004. We compared the clinical data of these patients with those from 47 patients who had right ventricular outflow tract reconstruction with a monocusp valve(monocusp ventricular outflow patch group : MVOP) and 22 patients who had a transannular patch repair without a monocusp valve(transannular patch group : TAP). Results : In the PVR group, 25 patients(81 percent) had trivial or mild pulmonary regurgitation in their early post operative echocardiogram. Only 12 patients(26 percent) in the MVOP group had mild pulmonary regurgitation; and no patient in the TAP group had it. Pulmonary valve function was good in 96 percent of the PVR group, 36 percent of the MVOP group, and none in the TAP group in early post-operative echocardiogram. Follow-up echocardiogram(1, 2, 3, 4 years later) of the MVOP and TAP groups showed moderate pulmonary regurgitation and severely decreased valve function in almost all cases. However, in the PVR group 54 percent(16/28), 50 percent(14/28), 37 percent(9/24), and 31 percent(5/16) of the patients had trivial or mild pulmonary regurgitation 1, 2, 3 and 4 years after operation, respectively. The valve function remained good in 80 percent(24/30), 64 percent(18/28), 57 percent(12/21), and 31 percent(5/16) of the patients 1, 2, 3 and 4 years after operation respectively. Conclusion : Pulmonary valve reconstruction is effective in reducing pulmonary regurgitation and right ventricular dilatation in the repair of TOF, even though regurgitation increases with time. Further study is needed to determine long-term results.

• Journal of Chest Surgery
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• v.32 no.3
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• pp.303-306
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• 1999

This is a genuine case report of the Ross operation without the use of homografts or heterografts in reconstruction of the right ventricular outflow tract. A 8-year-old boy with congenital aortic stenosis underwent aortic valve replacement with a pulmonary autograft and right ventricular outflow tract reconstruction with a pericardial conduit bearing autologous aortic monocusp. The postoperative echocardiography and cardiac angiography revealed good ventricular function and competent neoaortic valve. He has been followed up for 19 months.

• Journal of Chest Surgery
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• v.22 no.2
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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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• v.17 no.4
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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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• v.33 no.6
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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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• v.16 no.3
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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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• v.33 no.10
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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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• v.49 no.5
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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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• v.29 no.8
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• pp.822-827
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• 1996

Ross procedure is an ideal operative modality for diseased aortic valve especially in children, but homogrart for right ventricular outflow tract(RVOT) reconstruction is not easily available in Korea. We tried to perform REV procedure for RVOT reconstruction in 10 young piglets(15.3 $\pm$ 1.3 kg) In an attempt to e clude the use of homograft in Ross operation. 3 of them survived after operation and raised till adult pig(about 70 kg), then examined their pulmonary arteries and hearts. Without any stenotic residues in the great arteries, we only found the deformed monocusp patch with severe calcification, which deprived the adequate valve function, but kept the pig growing normally We are sure that this operative modality (REV + Ross procedure) could be extendedly applied to the diseased human aortic valve, but we need to develope the anti-calcification method for the heterograft patch.