• Title/Summary/Keyword: Survival duration

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Tetralogy of Fallot Associated with Pulmonary Atresia and Major Aortopulmonary Collateral Arteries -Comparison between the different surgical approaches- (폐동맥폐쇄와 주대동맥폐동맥혈관부행지를 동반한 활로씨사징증 -다양한 외과적 치료접근의 비교-)

  • 이정렬;양지혁;김용진;노준량
    • Journal of Chest Surgery
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    • v.32 no.5
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    • pp.471-479
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    • 1999
  • Background: Patients with tetralogy of Fallot(TOF), pulmonary atresia(PA) and major aortopulmonary collateral arteries(MAPCAs) have been managed by heterogenous surgical strategies. We have taken 5 different surgical approaches to this lesion. In this study, relative advantages and disadvantages of each strategy were discussed by analyzing our surgical results. Material and Method: Between January 1986 and June 1998, 50 patients aged 1 to 177(39${\pm}$31) months at a various morphologic spectrum of this lesion were treated at our institution. The groups, which were classified according to the initial approaches, consisted of one-stage unifocalization(A-1, n=9), staged unifocalization(A-2, n=11), right ventricle to pulmonary artery connection(RV-PA)(B-1, n=11), one-stage RV-PA plus unifocalization (B-2, n=11), and one-stage definitive repair(C, n=8). Morphologic charateristics, operative mortalities, and probabilities of definitive repair were compared between the groups and the causes of death, complications and the follow-up results were described. Result: Mean ages at the first operation were 57${\pm}$18(A-1), 42${\pm}$48(A-2), 18${\pm}$14(B-1), 52${\pm}$55(B-2), and 32${\pm}$34(C) months (p<0.05). Mean numbers of MAPCAs were least in group C (A-1=4.3${\pm}$1.0, A-2=4.5${\pm}$1.3, B-1=4.1${\pm}$1.9, B-2=4.1${\pm}$1.6, C=3.4${\pm}$1.8 : p<0.05). The ratios of the direct and the indirect MAPCAs in each group were not different between the grousps (A-1=91%/9%, A-2=78%/22%, B-1=80%/20%, B-2=80%/20%, C=81%/19% : p>0.05). Nineteen patients had more than 1 dependent MAPCAs. True pulmonary arteries were not present 13 patients and they were confluent in 29. A total of 101 operations were performed. Operative mortalities of initial procedures were 33%(3/9, A-1), 18%(2/11, A-2), 0%(0/11, B-1), 36%(5/11, B-2) and 13%(1/8, C) (p<0.05). Percentages of the definitive repair among the patients of each group were 22%(2/9, A-1), 18%(2/11, A-2). 45%(5/11, B-1), 27%(4/11, B-2), and 100%(8/8, C)(p<0.05). Hypoxic respiratory and cardiac failure(6), hypoxic encephalopathy(2) and sepsis(4) were the major causes of death. Phrenic nerve palsy occured in 5 patients. Thirty-one patients among the survivals have been followed up for a mean duration of 74${\pm}$42(3-145) months. One and five year actuarial survival rates were 73% and 73 %. Conclusion: In conclusion, Right ventricle to pulmonary artery connection used in patients with TOF/PA/MAPCA as an inital procedure appeared to be highly successful in enhancing the chance of satisfactory definitive repair without the significant surgical risks. One-stage total repair at an ealier age group could be performed safely with the resonable outcomes. Unifocalization approach, whether it was performed in a single stage or in the multiple stages, resulted in the high operative mortality and the lowest chance of definitive repair, however more tailored selection of the patients and the long follow-up is mandatory to prove the usefulness of this approach.

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The Clinical Application and Results of Palliative Damus-Kaye-Stansel Procedure (고식적 Damus-Kaye-Stansel 술식의 임상적 적용 및 결과)

  • Lim, Hong-Gook;Kim, Soo-Jin;Kim, Woong-Han;Hwang, Seong-Wook;Lee, Cheul;Shinn, Sung-Ho;Yie, Kil-Soo;Lee, Jae-Woong;Lee, Chang-Ha
    • 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.