BAI Song,LI Xiao feng,YUAN Feng,et al.Unifocalization repair for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.[J].Journal of Clinical Pediatric Surgery,2010,9(06):410.
单源化手术治疗肺动脉闭锁合并室间隔缺损及重要主肺动脉侧支血管
- Title:
- Unifocalization repair for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.
- Keywords:
- Heart Diseases/SU; Heart Defects; Congenital; Heart Septal Defects; Ventricular; Pulmonary Artery
- 文献标志码:
- A
- 摘要:
- 目的探讨肺动脉闭锁合并室间隔缺损及重要主肺动脉侧支血管的手术方式，评价其临床疗效。方法2008年7月至2010年8月本院采用单源化手术治疗肺动脉闭锁（PA）合并室间隔缺损（VSD）及重要主肺动脉侧支血管（MAPCAs）患儿8例。男3例，女5例，年龄4～72个月，中位年龄24个月，体重7.5～15.5kg，平均体重（11.2±2.4）kg。7例采取分期手术治疗，1例采取一期根治手术。结果全组患儿均存活。随诊6 ～ 20个月，1例一期根治手术患儿痊愈；7例分期手术患儿，临床紫绀症状均好转。其中3例肺动脉明显发育，进一步行二期根治手术治愈。结论单源化手术是治疗肺动脉闭锁合并室间隔缺损及重要主肺动脉侧支血管的有效方法，分期手术可取得良好的临床疗效。
- Abstract:
- ObjectiveTo review the opertion experience and outcome of unifocalization repairs for PA with VSD and MAPCAs.MethodsFrom Jul. 2008 to Aug. 2010, 8 patients (male 3, female 5) with age ranged from 5 to 72 (median 8.2) months and body weight ranged from 4.5 to 13 (mean 8.7±2.8) kg were treated with unifocalization repairs. One case underwent radical operation. The other 7 cases underwent a multistaged repair approach.ResultsThere were no deaths, all cases were being referred to the outpatient department for followup which lasted for 6 to 20 months, When reasonable pulmonary vascularization has been obtained, there were 3 cases were treated with complete repair with transventricular closure of the VSD and right ventricular outflow tract reconstruction.ConclusionsUnifocalization as a palliative operation is a useful method for patients PA with VSD and MAPCAs. Staged repair yields a relatively low mortality rate with good functional results.
参考文献/References:
1Ben DM,Shafi M.Unifocalization of major aortopulmonary collateral arteries in pulmonary atresia with ventricular septal defect is essential to achieve excellent outcomes irrespective of native pulmonary artery morphology\[J\].Thorac Cardiovasc Surg, 2009, 138: 1269—1275.2Haworth SG.Collateral arteries in pulmonary atresia with ventricular septal defect: a precarious blood supply\[J\].Br Heart J, 1980,44:5—13.3Gary K.OneStage Repair and Unifocalization for Pulmonary Atresia with Ventricular Septal Defect and Major Aortopulmonary Collateral Arteries in Early Infancy. 4Gary K. An overview of pulmonary atresia, ventricular septal defect, and multiple aorta pulmonary collateral arteries\[J\]. Pediatric Cardiology, 2009, 26: 65—70.5Sunil P,Frank L.Surgical Management of Pulmonary Atresia With Ventricular Septal Defect and Major Aortopulmonary Collaterals: A ProtocolBased Approach\[J\].Pediatr Cardic Surg Ann, 2009, 12:145—151.6SukWon Song,Han Ki Park.PA With VSD and MAPCA: 18Year Clinical Experience and Angiographic Followup of Major Aortopulmonary Collateral Arteries\[J\].Circ J, 2009, 73: 516—522.7Richard A，Jonas.Comprehensive Surgical Management of Congenital Heart Disease\[M\].First published, Oxford University Press Inc，2004, 440—456.8Nobuyuki Ishibashi,Toshiharu Shin,et al.Clinical results of staged repair with complete unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries\[J\].Cardiothoracic Surgery,2007, 32: 202—208.9Ishibashi N,Shin’oka T.Clinical results of staged repair with complete unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries\[J\].Eur J Cardiothorac Surg,2007,32: 202—208.
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备注/Memo
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