• 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.

• Journal of Chest Surgery
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• v.30 no.9
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• pp.927-931
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• 1997

Tricuspid regurgitation due to rupture of a chorda is a rare disease in newborns. Recently, we experienced one day old male with tricuspid regurgitation due to rupture of a chorda of anterior papillary muscle, and who had suffered from severe hypoxemia, acidosis, cyanosis, and bradycardia. Preoperative diagnosis was pulmonary atresia with intact ventricular s ptum, massive tricuspid regurgitation, and patent ductus arteriosus by echocardiogram, which demonstrated no flow through the pulmonic valve. At operation, the pulmonic valve was intact and a chorda of anterior papillary muscle was ruptured. Tricuspid regurgitation was corrected successfully with reconstruction of the chords. Postoperative course was complicated by pneumonia and sepsis, but the infant recovered and discharged at postoperative 20 days.

• Tuberculosis and Respiratory Diseases
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• v.40 no.2
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• pp.147-152
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• 1993

Background: There is evidence that platelet is activated in chronic obstructive pulmonary disease and activated platelet with injured endothelium contribute to the pathogenesis of pulmonary hypertension, prognostic factor of chronic obstructive pulmonary disease. So, we have investigated platelet function further in chronic obstructive pulmonary disease and effect of platelet activation on pulmonary hypertension. Method: We studied platelet aggregation ratio and alpha-granule products such as platelet factor 4(PF4) and beta-thromboglobulin (${\beta}$-TG) in control subjects and COPD without and with pulmonary hypertension subjects. Result: 1) The platelet aggregation ratio (PAR) was $0.99{\pm}0.04$ in control subjects, $0.98{\pm}0.05$ in COPD without pulmonary hypertension subjects and $0.89{\pm}0.08$ in COPD with pulmonary hypertension subjects. The platelet aggregation ratio of COPD subjects was tend to decrease than that of control subjects and the ratio of COPD with pulmonary hypertension subjects was significantly lower than that of control subjects. 2) The platelet factor 4 (PF4, IU/ml) was $4.7{\pm}1.2$ in control subjects, $18.6{\pm}4.9$ in COPD without pulmonary hypertension subjects and $57.2{\pm}12.7$ in COPD with pulmonary hypertension subjects. The level of COPD subjects was significantly higher than that of control subjects and the level of COPD with pulmonary hypertension subjects was significantly higher than that of COPD without pulmonary hypertension subjects. 3) The beta-thromboglobulin (${\beta}$-TG, IU/ml) was $34.4{\pm}5.8$ in control subjects, $80.4{\pm}18.1$ in COPD without pulmonary hypertension subjects and $93.0{\pm}14.0$ in COPD with pulmonary hypertension subjects. The level of COPD subjects was significantly higher than that of conrtrol subjects and the level of COPD with pulmonary hypertension subjects was tend to increase than that of COPD without pulmonary hypertension subjects. 4) There was no correlation between the clinical parameters and PAR, PF4 and ${\beta}$-TG but there was significant correlation among PAR, PF4 and ${\beta}$-TG. Conclusion: The platelet is activated in chronic obstructive pulmonary disease and the platelet of COPD with pulmonary hypertension is tend to be activated more than that of COPD without pulmonary hypertension. So, activated platelet may involve in the pathogenesis and maintenance of pulmonary hypertension in COPD subjects and modulation of platelet activity that might reduce pulmonary hypertension needs to be determined.

• 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.30 no.5
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• pp.524-527
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• 1997

Pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals is a rare and complex lesion in which great morphologic variability exists regarding the sources of pulmonary blood flow. We report a case of this disease with no true central pulmonary arteries in a 9-month-old-boy successfully treated by one-stage complete unifocalization and repair from a midline stemotomy approach.

• Journal of Chest Surgery
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• v.34 no.4
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• pp.361-364
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• 2001

자가 폐동맥 판막을 이용한 대동맥 판막 치환술을 항응고제 복용이 필요없고 내구성이 어느정도 입증되어 늘어나고 있으나, 자가 폐동맥 판막을 이용한 승모판막 치환술은 국내에 보고된 예가 없다. 53세 여자 환자로 류마티스성 승모판막 협차가 및 폐쇄부전, 삼첨판막 폐쇄부전, 만성 심방 세동, 그리고 자회전 관상동맥의 폐색등으로 진단받은 환자에서 자가 폐동맥 판막을 이용한 승모판막 치환술 및 maze 술식, 삼천판막 성형술, 관상동맥 우회수술을 시해하였다. 수술후 특별한 문제없이 회복하였으며 술후 시행한 심초음파 검사상 자가 폐동맥 판막의 이상 소견없이 잘 기능하고 있으며 항응고제 복용없이 잘 지내고 있다.

• Journal of Chest Surgery
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• v.35 no.5
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• pp.350-355
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• 2002

Background: Most of pulmonary regurgitation with or without stenosis appears to be well tolerated early after the repair of pulmonary outflow tract. However, it may result in symptomatic right ventricular dilatation, dysfunction and arrhythmias over a long period of time. We studied the early outcome of pulmonary valve replacement with tissue valves for patients with the above clinical features. Material and Method: Sixteen consecutive patients who underwent pulmonary valve replacement from September 1999 to February 2002 were reviewed(9 males and 7 females). The initial diagnoses included tetralogy of Fallot(n=11), and other congenital heart anomalies with pulmonary outflow obstruction(n=5). Carpentier-Edwards PERIMOUNT Pericardial Bioprostheses and Hancock porcine valves were used. The posterior two thirds of the bioprosthetic rim was placed on the native pulmonary valve annulus and the anterior one third was covered with a bovine pericardial patch. Preoperative pulmonary regurgitation was greater than moderate degree in 13 patients. Three patients had severe pulmonary stenosis. Tricuspid regurgitation was present in 12 patients. Result: Follow-up was complete with a mean duration of 15.8 $\pm$ 8.5months. There was no operative mortality. Cardiothoracic ratio was decreased from 66.0 $\pm$ 6.5% to 57.6 $\pm$ 4.5%(n=16, p=0.001). All patients remained in NYHA class I at the most recent follow-up (n=16, p=0.016). Pulmonary regurgitation was mild or absent in all patients. Tricuspid regurgitation was less than trivial in all patients. Conclusion: In this study we demonstrated that early pulmonary valve replacement for the residual pulmonary regurgitation with or without right ventricular dysfunction was a reasonal option. This technique led to reduce the heart size, decrease pulmonary regurgitation and tricuspid regurgitation as well as to improve the patients'functional status. However, a long term outcome should be cautiously investigated.

• Proceedings of the KTCVS Conference
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• 1995.10a
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• pp.73-73
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• 1995

• Tuberculosis and Respiratory Diseases
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• v.63 no.4
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• pp.346-352
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• 2007

Background: Pulmonary hypertension is one of the cardiovascular complications of in COPD. However, a diagnosis of pulmonary hypertension requires an invasive test, such as right heart catheterization. NT-proBNP is a cardiac hormone that is elevated when a cardiac volume or pressure overload is present. It was hypothesized that NT-proBNP might play a role in detecting of pulmonary hypertension in COPD patients. Method: The 31 COPD patients, who underwent all of NT-proBNP, echocardiography, and spirometry in Seoul National University Bundang Hospital during the period from November 2003 to July 2005, were retrospectively analyzed. Result: Of the 31 COPD patients, 9 patients had pulmonary hypertension. A significant positive correlation was observed between the NT-proBNP and pulmonary arterial pressure (r=0.589, p=0.002). However, there was no significant correlation observed between the $FEV_1$ and NT-proBNP and $FEV_1$ and pulmonary arterial pressure. Conclusion: NT-proBNP might indicate the presence of pulmonary hypertension in COPD patients.

• Journal of Chest Surgery
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• v.34 no.5
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• pp.407-409
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• 2001

기존의 중앙 단락술을 기술적으로 변형하여 두 명의 신생아를 수술하였다. 해부학적 진단은 심실 중격 결손을 동반한 폐동맥 동맥관 개존증이었다. 일차 수술에서 대동맥-단락간 문합을 측-측 문합 방식으로 하였다. 관찰기간 동안 양쪽 폐동맥과 주폐동맥은 잘 성장하였고 각각 10, 18개월에 Lecompte 술식을 시행하였다. 이 방법은 짧고 뒤틀림이 적은 직선주행의 단락을 만드는 장점을 가지고 있다.