• Journal of Chest Surgery
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• v.33 no.12
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• pp.963-967
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• 2000

정상심실중격의 폐동맥협착은 흔한 선천성 심장질환이지만 신생아기에 심한 증세를 보이는 위기적 폐동맥 협착은 드물며 예후도 대단히 나쁘다. 경피적 풍선 판막성형술의 발달과 함께 폐동맥협착의 외과적 치료는 더욱 줄어드는 추세이다. 본 증례는 정상심실중격의 위기적 폐동맥협착증으로 진단받은 생후 2일된 남자 신생아로 심한 청색증과 저산소증을 보여 응급실로 내원하였다. 환아는 산소공급 및 Prostaglandin E$_1$을 투여 후 동맥혈 산소 분압이 19 mmHg에서 54mmHg로 증가하였다. 경피적 풍선 판막성형술을 시도하였으나 유도도관(Guide wire)이 판막의 개구부를 통과하지 못하였고 시술도중 심낭내로 조영제가 고이는 소견을 보여 우심실 천공이 의심되었으므로 응급으로 정상체온의 체외순환하에서 폐동맥 절개후 폐동맥 판막절개술을 시행하였다. 수술 후 중환자실에서의 수술경과는 양호하였고 현재 수술 6개월째 외래 추적관찰중이다.

• Journal of Chest Surgery
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• v.34 no.7
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• pp.552-555
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• 2001

Noonan syndrome is characterized by a Turner-like phenotype and a normal karyotype associated with congenital abnormalities, such as short stature, variable mental retardation, hypertelorism, webbed neck, low posterior hair line, skeletal malformation and congenital cardiovascular defect. Two third of Noonan syndrome have cardiac anormalies, half with pulmonary stenosis. We have experienced two cases of pulmonary stenosis associated with other cardiac anomalies in Noonan syndrome. The first 31-year-old male patient had characteristic appearance of Noonan syndrome with severe infundibular pulmonic stenosis and patent foramen ovate. The second 28-year-old male patient had valvular and subvalvular Pulmonary stenosis with typical Noonan\`s face and stature. Pulmonary valvotomy and hypertrophied muscle bundles in the right ventricular cavity were resected in both cases. Patent foramen ovate was closed directly in the first case. Postoperative follow-up examinations revealed no symptoms and successful outcome.

• Journal of Chest Surgery
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• v.30 no.8
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• pp.747-751
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• 1997

We developed a subcutaneously adjustable new pulmonary artery banding device which can be easily tightened or released in patients with unstable postoperative hemodynamics. The banding device consists of stainless spring which is enveloped with PTFE, nd polyvinyl catheter(5F). And the adjuster consists of another polyvinyl tube with forming cap. We mark transluscent polyvinyl tube 1mm each in length. 6 dogs weighing 15 to 25 kg underwent banding of descending thoracic aorta with the device. At postoperative 1 and 2 day, the device was effectively banded and released. Even 3 months later, we noted effective banding with some degree of vessel injury. The preliminary animal study suggests that this new device may be applicable in patient with excessive pulmonary blood flow.

• Journal of Chest Surgery
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• v.42 no.3
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• pp.361-363
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• 2009

A 17-year-old male patient was referred with symptoms of dyspnea. Multi-detector computerized tomography (MDCT) and echocardiography evaluation revealed quadricuspid aortic and pulmonary valves, an atrial septal defect (ASD), and pulmonary stenosis. We closed the ASD using a bovine patch and performed a commissurotomy of the pulmonary valve. Quadricuspid semilunar valves are very rare congenital abnormalities that are reported to occur nine times more frequently in the pulmonic valve than in the aortic valve. According to the Hurwitz Roberts classification, the aortic valve was type A, and the pulmonic valve was type B. The aortic valve normal function, but the pulmonic valve was stenotic and had abnormal function.

• Journal of Chest Surgery
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• v.35 no.6
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• pp.439-448
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• 2002

Pulmonary artery banding (PAB) in the functional univentricular heart (UVH) is a palliative procedure for staging toward the Fontan procedure; however, it is known to be a risk factor. Material and method： The records of all 37 patients with functional UVHs who underwent surgical palliation using PAB between September 1989 and August 1999 were reviewed retrospectively. We investigated the aortic arch obstruction, the development and progression of subaortic stenosis after PAB, and risk factor of mortality according to surgical method. Result： In 37 neonates and infants with single ventricular physiology, aortic arch obstruction was combined in 7. There were 6 early deaths (16.2%) after PAB and 3 late deaths (8.1%) after Fontan operation. The actuarial overall survival including early mortality at 3 and 5 years were 80.7$\pm$6.6%, 72.2$\pm$8.2% respectively. Among 31 patients who survived PAB, 27 patients (87.1 %) could become candidates for Fontan operation； 22 patients (71.0%) completed Fontan operation with 3 deaths and 5 were waiting bidirectional cavopulmonary shunt(BCPS) or Fontan operation (follow-up mean 4.5 year, minimal 2 year). Subaortic stenosis developed in 8 patients after PAB (8/29, 27.6%)； 3 cases in the patients without arch anomaly (3/22, 13.6%) and 5 in those with arch anomaly (5/7, 71.4%). The subaortic stenosis was managed with Damus-Kaye-Stansel procedure (DKS) in 6 patients without operative mortality and conal septum resection in 2 without long-term survivor. Analysis of risk factors established that aortic arch obstruction was strongly associated with subaortic stenosis (p＜0.001). The only risk factor of late mortality was Fontan procedure without staged palliation by BCPS (p=0.001). Conclusion： PAB is effective as an initial palliative step in functional UVH. And the high risk group of patients with aortic obstruction can undergo effective short-term PAB as an initial palliative step, with subsequent DKS for subaortic stenosis. This strategy, initial PAB and careful surveillance, and early relief of subaortic stenosis can maintain acceptable anatomy and hemodynamics for later Fontan procedures.

• Journal of Chest Surgery
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• v.31 no.9
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• pp.861-866
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• 1998

Background: Pulmonary atresia (PA) with ventricular septal defect has various morphology of pulmonary arteries and pulmonary blood flow sources, so pulmonary arterial hypoplasia and arborization abnormality make this anomaly difficult to manage surgically. In cases associated with juxtaductal stenosis, we evaluated the change of the pulmonary arterial and juxtaductal stenotic site after shunt operations, and would like to find useful information in surgical planning and methodology of these patients. Material and Method: Among 59 cases diagnosed as PA with ventricular septal defect associated with juxtaductal stenosis, 29 cases who had cardiac catheterization before and after shunt operation were selected from July, 1991 to July, 1996. In 10 cases of right shunt operation(Group I) and 19 cases of left shunt operation (Group II), the diameters of the descending aorta, both pulmonary arteries, and the juxtaductal stenosis site were measured before and after the shunt operation. Result: In both Group I and II, the pre- and postoperative ratio of diameters of the ipsilateral pulmonary artery to the descending aorta was from 0.78${\pm}$0.31 units to 1.01${\pm}$0.26 units and from 0.67${\pm}$0.18 units to 0.84${\pm}$0.27 units respectively, showing a signigicant increase. The contralateral pulmonary artery index was increased from 0.92${\pm}$0.28 units to 1.05${\pm}$0.15 units and from 0.94${\pm}$0.27 units to 1.08${\pm}$0.37 units respectively, but could not be confirmed statistically. In both groups, the change of juxtaductal stenosis showed an aggravating tendency but of no statistical significance from 0.43${\pm}$0.27 units to 0.39${\pm}$0.25 units and from 0.32${\pm}$0.10 units to 0.30${\pm}$0.16 units respectively, and we experienced 2 total obstruction in Group II. Because the increased pulmonary blood flow by shunt operation has a favorable effect to the pulmonary arterial growth, the shunt operation is a recommended treatment in patients with hypoplastic pulmonary arteries. But in PA with ventricular septal defects, the change of juxtaductal stenosis is very important. In conclusion, the growth of ipsilateral (shunt site) pulmonary artery was promoted by shunt operation, but there is a tendency for the juxtaductal stenosis to be aggravated. And we experienced 2 total obstruction in Group II. Conclusion: Thus, in cases operated with shunt method, much careful postoperative follow up study including angiographic evaluation is needed, and after the shunt operation on the side of pulmonary artery associated with juxtaductal stenosis, early precise planning for total correction is recommended.

• Journal of Veterinary Clinics
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• v.26 no.1
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• pp.48-53
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• 2009

A 4-year-old intact male Shih Tzu dog (5.4 kg of body weight) was referred with primary complaints of heart murmur and exercise intolerance. Diagnostic studies found severe valvular pulmonic stenosis (peak velocity of 6.4 m/s, pressure gradient 165 mmHg). The dog was treated with 3 cm (length) ${\times}$ 1.5 cm (diameter) of Palmaz biliary stent. The outcome of stent placement was favorable and remarkably reduced the severity of PS (6.4 m/s to 3.0 m/s of peak velocity). Clinical condition was remarkably improved with the absence of cardiac murmur, although mild pulmonic regurgitation existed from the day of stent placement. To our best knowledge, this case is the first clinical trial for treating PS with intravascular stent in Korea.

• Clinical and Experimental Pediatrics
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• v.46 no.1
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• pp.67-75
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• 2003

Purpose : Intravascular stent implantation for the treatment of postoperative branch pulmonary artery(PA) stenosis has been used successfully. However, the cross sectional area of contralateral branch PA does not regress in spite of the successful dilation of the stenotic branch PA after stent implantation. We analyzed the morphologic and hemodynamic factors on the size of branch PA after successful stent implantation. Methods : The subjects in our study were 23 children who had undergone stent implantaion from Jan. 1995 to Jul. 2002 in the Division of Yonsei Pediatric Cardiology. We evaluated the cross sectional area index(CSAI) of branch PA before and after stent implantation at follow-up catheterization. We also investigated factors such as residual pulmonary stenosis, pulmonary regurgitation(PR), systolic pressure of right ventricle, and lung perfusion scan. Results : The CSAI of the RPA without stenosis changed from mean $238{\pm}17mm^2/BSA$ to mean $249{\pm}20mm^2/BSA$(P=0.47), but didn't regress. The CSAI of the LPA with stenosis was increased effectively by stent implantation from the mean $102{\pm}12mm^2/BSA$ to mean $125{\pm}11mm^2/BSA$(P< 0.05). At follow up after stent implantation, the CSAI of PA is correlated with the residual PR fraction after stent implantation. Conclusion : In a group with increased residual PR, CSAI of RPA was found to be significantly increased between the pre- and post-stages of stent implantation. So, we suspect that the pulmonary regurgitation remaining after right ventricle outlet tract(RVOT) dilatation surgery is correlated with the increase in CSAI of RPA.

• Journal of Chest Surgery
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• v.36 no.4
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• pp.255-260
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• 2003

Background: Stenosis of the left pulmonary artery (LPA) after repair of tetralogy of Fallot (TOF) is troublesome. A new technique of LPA angioplasty using an autologous MPA flap was performed in patients with TOF Material and Method: From October 1998 to January 2001, 24 patients (median age; 10 months, range; 4 to 145 months) underwent total correction of TOF with LPA angioplasty using the autologous MPA flap. Five patients underwent pulmonary angioplasty without any patch over the MPA and LPA. The patches were required to enlarge only the MPA in 4 patients, and transannular RVOT widening was performed in 15. Result: There were no operative or late deaths. During follow-up (range: 6~42 months), reoperation for LPA stenosis was not required in any patients, but balloon angioplasty for branch pulmonary artery stenosis was performed in 3 patients. Echocardiography and CT angiography at the recent follow-up showed an obtuse angle between the MPA and LPA. Conclusion: Although further follow-up is needed, the angioplasty using the autologous MPA flap can be easily performed, avoiding patch-related complications, and allowing growth of the MPA flap. This angioplasty technique creates a more natural and obtuse angle between the MPA and LPA, which can minimize kinking of the LPA, especially in the patients who underwent transannular patch widening.

• 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.