• 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.29 no.10
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• pp.1095-1101
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• 1996

From July 1983 to June 1995, 95 consecutive patients with mitral stenosis were treated surgically in the Department of Thoracic and Cardiovascular Surgery, Chonbuk national University Hospital, mitral valve replacement(MVR) in 62 patients and open mitral commissurotomy(OMC) in 33 patients. Mitral stenosis combined with coronary artery disease, with aortic valve disease, or wish mitral valvular Insufficiency, were excluded from this study. Surgical procedures for mitral stenosis were evaluated, according to complication, reoperation, mor- tality, nd functional change at mid- and long-term postoperative period. Cardiothoracic ratio in the MVR group was greater than the OMC group(0.59 $\pm$0.07 in MVR, 0.53 $\pm$0. 07 in OMC, p<0.05), but other variables(age, sex, MYHA functional classification, EKG finding, echocardiographic finding) did not show significant difference between two groups in the preoperative periods. Even though pathologic valvular lesion(Sellor's pathologic type m: 35 in MVR, 13 in OMC) and valvular calcification(35 in MVR, 11 in OMC) were severe in the MVR group(p=0.001) at intraoperative observation, OMC was possible in 11 patients(23.9%) among 46 patients with valvular calcification and in 13 patients(27.1 %) among 61 patients with Sellor's pathologic type IH . There was no significant difference in early and late mortality, actuarial survival(75% in MVR, 87.6% in OMC at 12 year), but early and late hemorrhagic, thromboembolic complications in the MVR group were greater than in the OMC. Functional changes in NYHA functional classification, EKG lEnding, cardiothoraclc ratio, and echocardiographic finding(EF, LVIDS, LWDd, LAD) did not differ between two groups in mid- and long-term postoperative periods. We conclude that our efforts for preservation of the native valve would be continued, because hemorrhagic and thromboembolic complications in the MVR were greater than in the OMC, and OMC was possible even in patients with severely stenotic and calcified mitral valve, although there was no sis-nificant difference in the functional change, mortality, and survival between the M VR and OMC.

• Journal of Chest Surgery
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• v.30 no.2
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• pp.152-157
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• 1997

From August 1986 until June 1995, single aortic valve replacement was performed in 65 patients at the Chonnam National University Hospital. worthy-eight were male and 17 were female patients, ranging from 19 to 68 years of age(median : 43 years). The causes of the valve lesions were rheumatic in 29 patients (44.6%), bicuspid aortic valve in 6 patients (6.2%), endocarditis in 6 patients(6.2%), unknown in others. Concomitant surgical procedures were performed in 10 patients : repair of congenital defect in 5, pericardiectomy in 1, coronary artery bypass grafting in 1, noncoronary sinus plication in 1, Valsalva sinus aneurysmectomy in 1, subaortic membrane resection in 1 Used valves were 51. Jude-Medical valve in 42, Duromedics valve in 22, Bjork-Shiley valve in 2, Carpentier-Edward valve in 1. There were 3 hospital deaths (4.6%), and 2 late deaths (3.2%). Follow-up was 95.2% complete. The 10-year acturlal survival rate was 85.3%. Postoperative complications were low cardiac utput in 8, arrythmia in 5, valve related hemolysis in 1, cerebral infarction in 1, and gastrointestinal bleeding in 2. Reoperation was performed in 4 for surgical bleeding, in 3 for paravalvular leak. The mean improvement in New York Heart Association functional class is from 2.79 $\pm$ 0.66 preoperatively to 1.25 $\pm$ 0.49 postoperatively(p < 0.001) The change of cardiothoracic ratio from preoperative to postoperative is 0.57 $\pm$ 0.06 to 0.54 $\pm$ 0.05 (p < 0.05). The left ventricular ejection fraction change is not significant perioperatively. There are no mechanical failures. This early and intermediate-term follow-up suggests that in adults in whom valve repair is not possible, the mechanical valve is a reliable and durable prosthesis with good hemodynamic function and a low rate of thromboembolic event.

• Journal of Chest Surgery
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• v.30 no.9
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• pp.891-898
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• 1997

Between May 1984 and January 1996, 130 patients were replaced cardiac valve using 150 St. Jude Medical prosthetic valves(42 aortic, 68 mitral, 20 aortic and mitral valve replycements). Follow-up was 97.6% complete. The early mortality rate was 5.4%, and late mortality rate was 4.9%. The valve-related late mortality rate was 3.3%. Of late complications, there were 6 anticoagulant related hemorrhages, 4 thromboembolisms and 1 paravalvular leakage. Linearized rates of late complication and valve-related late mortality were as follows: total late complications, .1.68o per patient-year: anticoagulant related hemorrhages, 0.92% per patient-year: thromboembolism, 0.61% per patient-year: paravalvular leakage, 0.15% per patient-year: reoperation, 0.15% per patient-year: and valve-related late mortalities, 0.61% per patient-year. Actuar al event free rate at 10 years was 87.4 $\pm$ 3.2%. The overall actuarial survival rate was 90.4$\pm$2.7% at 5 years, 87.5$\pm$3.3% at 10 years. Ninety eight percent of the survivors were in the New York Heart Association functional class I or II at the end of follow-up. There was significant improvement of cardiothoracic ratio. In conclusion, this study suggests the excellent durability of the St. Jude Medical Heart valve and remarkable functional benefit for the majority of the patients. However, prosthesisrelated complications are still common. Outcome is strongly related to the patient's preoperative cardiac condition and to the adequacy of anticoagulation control.

• 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.

• Clinical and Experimental Pediatrics
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• v.48 no.12
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• pp.1362-1369
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• 2005

Purpose : This study aimed to investigate the correlation between the plasma level of N-terminal pro-B-type natriuretic peptide(pro-BNP) and several known risk factors influencing outcomes after Fontan operations, and to assess whether pro-BNP levels can be used as predictive risk factors in Fontan operations. Methods : Plasma pro-BNP concentrations were measured in 35 patients with complex cardiac anomalies before catheterization. Cardiac catheterization was performed in all subjects. Mean right atrium pressure, mean pulmonary artery pressure(PAP), and ventricular end-diastolic pressure(EDP) were obtained. Cardiac output and pulmonary vascular resistance were calculated by Fick method. Results : Plasma pro-BNP levels exhibited statistically significant positive correlations with mean PAP(r=0.70, P<0.001), pulmonary vascular resistance(r=0.57, P<0.001), RVEDP(r=0.63, P<0.001), LVEDP(r=0.74, P<0.001), and cardiothoracic ratio(r=0.71, P<0.001). The area under the ROC curve using pro-BNP level to differentiate risk groups in Fontan operations was high : 0.868(95 percent CI, 0.712-1.023, P<0.01). The cutoff value of pro-BNP concentrations for the detection of risk groups in Fontan operations was determined to be 332.4 pg/mL(sensitivity 83.3 percent, specificity 82.7 percent). Conclusion : These data suggest that plasma pro-BNP levels may be used as a predictive risk factor in Fontan operations, and as a guide to determine the mode of therapy during follow-up after Fontan operations.

• Journal of Chest Surgery
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• v.41 no.1
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• pp.1-11
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• 2008

Background: The Damus-Kaye-Stansel (DKS) procedure is a proximal MPA-ascending aorta anastomosis used to relieve systemic ventricular outflow tract obstructions (SVOTO) and pulmonary hypertension. The purpose of this study was to review the indications and outcomes of the DKS procedure, including the DKS pathway and semilunar valve function. Material and Method: A retrospective review of 28 patients who underwent a DKS procedure between May 1994 and April 2006 was performed. The median age at operation was 5.3 months ($13\;days{\sim}38.1\;months$) and body weight was 5.0 kg ($2.9{\sim}13.5\;kg$). Preoperative pressure gradients were $25.3{\pm}15.7\;mmHg$ ($10{\sim}60\;mmHg$). Eighteen patients underwent a preliminary pulmonary artery banding as an initial palliation. Preoperative main diagnoses were double outlet right ventricle in 9 patients, double inlet left ventricle with ventriculoarterial discordance in 6,. another functional univentricular heart in 5, Criss-cross heart in 4, complete atrioventricular septal defect in 3, and hypoplastic left heart variant in 1. DKS techniques included end-to-side anastomosis with patch augmentation in 14 patients, classical end-to-side anastomosis in 6, Lamberti method (double-barrel) in 3, and others in 5. The bidirectional cavopulmonary shunt and Fontan procedure were concomitantly performed in 6 and 2 patients, respectively. Result: There were 4 hospital deaths (14.3%), and 3 late deaths (12.5%) with a follow-up duration of $62.7{\pm}38.9$ months ($3.3{\sim}128.1$ months). Kaplan-Meier estimated actuarial survival was $71.9%{\pm}9.3%$ at 10 years. Multivariate analysis showed right ventricle type single ventricle (hazard ratio=13.960, p=0.004) and the DKS procedure as initial operation (hazard ratio=6.767, p=0.042) as significant mortality risk factors. Four patients underwent staged biventricular repair and 13 received Fontan completion. No SVOTO was detected after the procedure by either cardiac catheterization or echocardiography except in one patient. There was no semiulnar valve regurgitation (>Gr II) or semilunar valve-related reoperation, but one patient (3.6%) who underwent classical end-to-side anastomosis needed reoperation for pulmonary artery stenosis caused by compression of the enlarged DKS pathway. The freedom from reoperation for the DKS pathway and semilunar valve was 87.5% at 10 years after operation. Conclusion: The DKS procedure can improve the management of SVOTO, and facilitate the selected patients who are high risk for biventricular repair just after birth to undergo successful staged biventricular repair. Preliminary pulmonary artery banding is a safe and effective procedure that improves the likelihood of successful DKS by decreasing pulmonary vascular resistance. The long-term outcome of the DKS procedure for semilunar valve function, DKS pathway, and relief of SVOTO is satisfactory.