Surgical management of the infant with coarctation of the aorta and ventricular septal defect.

Clinical and cardiac catheterization data were collected from 39 infants with coarctation of the aorta and ventricular septal defect, 31 of whom were initially managed only by surgical repair of coarctation. Data were analyzed to determine mortality, morbidity, outcome and factors that might predict survival or the need for septal defect closure. Of the eight patients who did not require surgical treatment before 3 months of age, seven underwent coarctation repair alone at a mean age of 2.3 years. Of the 23 infants managed with coarctation repair alone, before age 3 months, 9 needed no additional surgical treatment and 6 required early and 8 required late repair of the ventricular septal defect. Seven infants underwent coarctation repair and simultaneous pulmonary artery banding and one eventually required debanding after spontaneous closure of the septal defect. The overall mortality rate in this series was 10.3% (mean follow-up time 5.7 years). Of 39 infants, 16 (41%) never required a second operation for ventricular septal defect closure. For patients who had only coarctation or coarctation repair with pulmonary artery banding at less than 3 months of age, ventricular septal defect size was categorized as small (less than 0.5 cm/m2), moderate (less than 1 cm/m2) or large (greater than 1 cm/m2) on the basis of defect size at operative repair or echocardiographic or angiographic assessment. Defect size did not necessarily correlate with the need for operative repair.(ABSTRACT TRUNCATED AT 250 WORDS)     

Congenital heart disease: diagnostic approaches, surgical indications and operative results

Our management policies of the main congenital cardiac defects which require emergency surgical intervention in neonate and infancy were reported. Total anomalous pulmonary venous drainage (TAPVD): Recent refinements in two-dimensional (2-D) echocardiography have made it possible to operate for this anomaly using only this modality. When a combination of coarctation is suspected and/or the location of the drainage of the pulmonary vein is obscure, catheterization and angiocardiography are added for detailed diagnosis. It is, however, our policy not to perform right-sided angiocardiography for patients younger than three months of age. Symptomatic cases are operated urgently, although not always on an emergency basis. In our experience, a 12 approximately 24 hour delay with intensive cardiorespiratory and metabolic care may improve the preoperative conditions considerably and increase the chances of a successful surgical repair. Coarctation of the aorta (CoA) and interruption of the aortic arch (IAA): Diagnosis of CoA by echocardiography and aortography by radial artery injection is well established. In neonates and infants with CoA or CoA + patent ductus arteriosus (PDA) and/or ventricular septal defect (VSD), emergency repair of coarctation (usually with subclavian flap aortoplasty) without pulmonary artery banding (PAB) is undertaken on the day of established diagnosis. If a combination of complex cardiac anomalies such as transposition of the great arteries (TGA) and Taussig-Bing anomaly is suspected, catheterization and angiocardiography are added for the detailed diagnosis. Repair of coarctation combined with PAB has been our choice of procedure in these infants. If IAA is suspected, catheterizaton, angiographic and detailed echocardigraphic studies are performed to define the precise anatomy of the aortic arch and associated intracardiac lesions, paying particular attention to the left ventricular outflow tract, as soon as such patients become clinically stable by intensive medical treatment using prostaglandin E1 (PGE1), digitalis and diuretics. Once the diagnosis is established surgical treatment should be carried out without delay. Initial palliation by aortic arch reconstruction with PAB followed by two-stage definitive intracardiac repair have been our choice of procedure in neonates and infants with IAA without severe left ventricular outflow tract obstruction.(ABSTRACT TRUNCATED AT 400 WORDS)     

Coarctation of the aorta in infancy

Twenty-five infants under 1 year of age (mean weight 3.4 kg) underwent repair of coarctation of the aorta between the years 1965 and 1982. Three patients had coarctation only, three had coarctation with patent ductus arteriosus (PDA), and 19 had associated intracardiac anomalies. Eleven patients underwent resection of the aorta and end-to-end anastomosis. Eight had subclavian flap arterioplasty, five had patch graft arterioplasty, and one had subclavian-to-aortic anastomosis. Additional procedures were performed on seven patients: banding of the pulmonary artery on one, repair of total anomalous pulmonary venous drainage on one, mitral valve replacement on one, aortic valvotomy on one, and aortic valvotomy plus pulmonary artery banding on one. Twenty-one survived the operation. All patients who died had associated intracardiac anomalies. The 21 survivors have been followed from 3 months to 13 years, with three late deaths that were associated with intracardiac anomalies. Five of the survivors underwent additional second operations: one had repair of the re-coarctation, one had replacement of the prosthetic mitral valve, one had aortic valvotomy, and two had pulmonary artery debanding and closure of a ventricular septal defect. Two of the 18 surviving patients have mild hypertension associated with a residual gradient. The others are in good health.     

6kg以下小婴儿主动脉缩窄合并室间隔缺损的外科矫治

目的 :探讨新生儿、小婴儿主动脉缩窄 (CoA)合并室间隔缺损 (VSD)的外科治疗方法。方法 :1998年 12月至 2 0 0 3年 7月共收治 9例 6kg以下CoA合并VSD患儿 ,其中新生儿 3例。一期手术 3例 ,分期手术 3例 ,仅做主动脉缩窄成形术 3例 ,其中 1例加做肺动脉环缩术。 6例采用人造血管补片主动脉成形术 ,3例采用左锁骨下动脉翻转主动脉成形术。结果 :全组患儿无死亡 ,随访 8～ 36个月 ,心功能改善明显 ,超声心动图检查提示无主动脉瘤样扩张或主动脉再狭窄。结论 :大多数患儿均可采用体外循环下一期根治CoA和VSD ,但是对于VSD为限制性及不能耐受体外循环的患儿可采用二期手术。一期手术选择左外侧和正中双切口方法安全有效。     

Neonatal anatomical repair of transposition of great vessels associated with atrial septal defect. Apropos of 42 cases

The good results obtained by anatomic correction of simple transposition of the great arteries (TGA) in the neonatal period have incited some surgical teams to widen the indications to neonates with TGA associated with ventricular septal defect (VSD). The classical management of these patients is a two stage procedure: banding of the pulmonary artery followed by detransposition, which carries a certain risk. Between January 1985 and June 1990, 42 neonates with TGA and VSD underwent a combined procedure consisting in anatomic correction of the TGA and closure of the VSD. The average age of these patients was 16 days, and the average weight was 3.3 kg. Ten patients had coarctation and 6 underwent a complete one stage correction by an anterior approach. The surgical technique consisted in closing the VSD from the right atrium in 20 patients, from the right ventricle in 11 patients and from the pulmonary artery in 11 patients, associated with detransposition of the great arteries and coronary artery reimplantation. Three children died in the preoperative period (7.1%). In two cases, death was related to malposition of the coronary artery. The third fatality was the result of haemorrhage. There has been one late death three years after surgery. Four patients have been reoperated for stenosis of the right ventricular outflow tract (1 case), recurrence of coarctation (2 cases) and stenosis of the superior vena cava (1 case) and have survived. All patients were followed up for an average period of 26.4 +/- 19 months. They are all in the NYHA Class I without treatment. One patient has mild aortic regurgitation.(ABSTRACT TRUNCATED AT 250 WORDS)     

Surgical treatment of ventricular septal defect in the first year of life]

Forty six infants with a large ventricular septal defect (VSD) underwent surgical treatment during the first 12 months of life. Forty three patients ranging in age from 3 to 12 months (mean age 10.4 months) and weighting from 3.0 to 8.2 kg (mean weight 6.8 kg) had primary surgical closure of their VSD. All infants were below the third percentile for weight preoperatively. In 40 patients (93%) the defect was closed through the right atrium. Three patients (7.0%) died in the early postoperative period. Surgically induced heart block occurred in one patient (2.3%). Late results were assessed in 29 surgical survivors (mean follow-up 26 months). There was no late mortality. Relief of congestive heart failure was prompt in all patients following closure of VSD. Right bundle branch block with left anterior hemiblock developed in 5 patients (17.2%), and right bundle branch block alone in 10 patients (34.5%). Three patients (mean age 4.3 months, mean weight 2.7 kg) underwent initial palliative pulmonary artery banding (PAB). There were no operative or late mortality. Closure of VSD and pulmonary artery debanding was performed in two of these patients, with no mortality. Prior to intracardiac correction the pulmonary artery pressure distal to the band was normal; no band related complications were found. Early primary closure is the treatment of choice for symptomatic infants with large VSDs. In particular circumstances, however, PAB may provide effective palliation.     

Enlarging angioplasty of the aortic arch with an pulmonary artery autograft patch. Interest in a single-stage repair of the syndrome of coarctation and interruption of the aortic arch]

Single stage repair of syndromes of coarctation and interruption of the aortic arch is a routine procedure in many surgical centres with good immediate results. The classical technique of aortic repair is based on the principles of Crafoord's extended resection anastomosis. Recoarctation is not an unusual long-term complication. A technique of enlarging angioplasty of the aorta using a patch of pulmonary artery has been developed and used in 22 neonates with obstruction of the aortic arch associated with ventricular septal defect with an average age and body weight of 15 days and 2.9 Kg respectively. The ventricular septal defect was closed surgically during the same procedure. Total circulatory arrest was not used in these children and all had aortic repairs with selective cerebral perfusion with moderate hypothermia (28-30 degrees C). This technique was used without any procedure-related early morbidity. No early or late deaths were observed in this series. Two patients were reoperated during the first year after the initial procedure: one for residual ventricular septal defect and the other for supraventricular pulmonary stenosis. Two patients, one of whom was reoperated, developed supraventricular pulmonary stenosis with a gradient of over 60 mmHg. These stenoses were observed in the first cases operated and were essentially due to the technique of pulmonary artery reconstruction. Over a median follow-up period of 10 months, no recoarctations were observed: the Doppler ultrasound study showed an isolated mean systolic gradient of 6 +/- 12 mmHg. The authors conclude that angioplasty of the aortic arch with an enlarging patch of pulmonary artery autograft during single stage surgery of syndromes of coarctation and interruption of the aortic arch provides a harmonious and durable repair of the aortic arch.     

Surgical management of coarctation of aorta with ventricular septal defect. Multivariate analysis.

Optimal management of coarctation with ventricular septal defect is difficult. Should one treat the coarctation, the ventricular septal defect, or both? This dilemma was investigated by reviewing 39 successive patients, aged less than 4 months, undergoing coarctation repair without pulmonary artery banding. Twelve hospital deaths occurred. Ventricular septal defect size was graded "blind" according to preoperative angiocardiographic and haemodynamic findings. Of 14 patients classified as having a large ventricular septal defect, necropsy and operative findings in eight showed defects 7 to 12 mm in diameter. Factors associated univariately with significantly increased mortality were young age, raised atrial and ventricular end-diastolic pressures, low weight, high admission blood urea, preoperative ventilation, and a large ventricular septal defect. All but the last two were also closely associated with each other. A jack-knifed discriminant function based upon ventricular septal defect size, blood urea, pulmonary venous oxygen content, and inferior caval oxygen saturation correctly predicted outcome in 78.9% of patients. Combinations of these four giving a probability greater than 0.9 of survival were rare with blood ureas above 8 mmol/litre. Raised blood urea was associated with low descending aortic pressure and subsequent dialysis. Only patients with a large ventricular septal defect stand to benefit from pulmonary artery banding at initial operation. Preoperative treatment to increase renal blood flow (prostaglandins, dopamine) may improve overall survival.     

Surgical correction of ventricular septal defects treated by banding the pulmonary artery. Apropos of 38 cases]

The authors report their experience with two-stage surgical treatment of severe ventricular septal defects in babies: initial banding of the pulmonary artery, followed by closure of the ventricular septal defect and removal of the band by an open-heart technique. Out of 162 operations to band the pulmonary artery, 90 were for cases of ventricular septal defect. The mortality was low (9.1%) when there was a ventricular septal defect with or without a patent ductus. It is considerably higher (31.4%) when there is a coincident coarctation of the aortic isthmus. So far 38 children have had the second operation, with 2 deaths. The authors have studied the surgical problems and the long-term results (6 months to 3 years follow-up) in the first 29 patients undergoing surgery. Although the results from this two-stage operation are good, when the authors take into account their current experience with one-stage closure of ventricular septal defects in the babies, they feel that this latter technique is to be preferred. Reservations must, however, be expressed when the child is very young (Weighing 3.5 kg or less), when there are multiple defects between the ventricles, and when the defect is severe, with a combination of VSD, patent ductus and coarctation of the aorta, and sometimes when the opertion must be carried out as an emergency.     

Value of dacron aortoplasty in the treatment of coarctation of the aorta during the 1st six months of life]

Between September 1st 1974 and June 1st 1976, 12 infants under the age of 6 months have been operated on by a dacron patch aortoplasty for coarctation of the aorta. The associated lesions were a patent ductus arteriosus in each case, a VSD in 8 cases, and a trans-position of the great arteries in 5 cases. A pulmonary artery banding was performed with the aortoplasty in 8 cases, and a Blalock-Hanlon operation in one instance. There were two operative deaths (17 p. 100), amongst them one in a 1400 g premature infant, and a late death (3rd month). Two infants have a clinical aspect of recoarctation. In four infants, the post-operative hemodynamic and angiographic study carried out before the treatment of associated intra-cardiac lesions, shows a good result of the coarctation repair. This particular technique, although not performed as a routine in the infant with coarctation of the aorta, seems to be of interest in the most severe forms of the disease, with diffuse isthmus narrowing and intra-cardiac defects.     

Novel pulmonary artery banding followed by transcatheter balloon dilatation in treatment of complex congenital heart defects

We present a novel technique of pulmonary arterial banding using a Gore-Tex strip secured by 7-0 Prolene suture as part of surgical treatment in 2 neonates with aortic coarctation and ventricular septal defects. Successful percutaneous balloon angioplasty of the main pulmonary artery in the area of pulmonary arterial band was performed at 12 and 26 months of age in one of the patients and at 3.5 months of age in the other patient. Peak systolic pressure drop across the area of previous pulmonary artery banding was 30 mmHg and 25 mmHg at 65 and 30 months of age, respectively.     

Hybrid方法建立肺血减少型先天性心脏病幼猪动物模型

目的 研究肺血减少型先天性心脏病(先心病)肺血管发育的病理生理过程及其相关调节机制,探讨肺血减少型先心病幼猪动物模型构建的可行性方法.方法 采用出生1～2个月的幼猪共20只,按照购入时的编号顺序(1～20),随机分为3组:(1)对照组(C组,n=6),右胸前外侧切口造成一过性肺血减少;(2)轻中度狭窄组(T1组,n=7),右胸前外侧切口经右心房表面送入球囊扩张器行人工房间隔造口+肺动脉Banding环缩术,术中收缩期肺动脉环缩处压差(systolic trans pulmonary artery banding pressure,Trans-PABP)20～30 mm Hg(1 mm Hg=0.133 kPa);(3)重度狭窄组(T2组,n=7),术中Trans-PABP≥30～50 mm Hg.术后1个月行64排CT扫描评估,2个月二次开胸手术测定动脉血气分析及血常规,测量各组血管直径及Trans-PABP,处死动物切取心肺组织测量房间隔缺损(ASD)和Banding环直径.结果 C组动物因麻醉意外,术后10 h死于呼吸衰竭1例.T1组术后21 d死于肠梗阻、肠坏死1例.T2组死亡2例,分别于术后24 b和39 d因急性和慢性右心功能衰竭死亡.T1和T2组存活动物房间隔造口+肺动脉环缩术均获得成功.术后超声测定T1和T2组房缺大小分别为(8.0±0.5)mm、(8.9±1.4)mm(P＞0.05),Trans-PABP术后至2个月持续显著增加,T1组由(19.1±5.6)mm Hg增加至(24.1±3.0)mm Hg(P＜0.01),T2组由(34.2±3.9)mm Hg增加至(43.6±6.4)mm Hg(P＜0.01).术前三组间氧分压(PaO2)和血细胞比容(Hct)差异均无统计学意义.术后2个月T1、T2组PaO2显著低于C组,且T2组低于T1,T1、T2组Hct显著高于C组,且T2组高于T1组(P＜0.05).64排CT扫描示T1组主动脉直径(AOD)明显低于C组,T1、T2组肺动脉环缩处直径显著低于各自的AOD(P＜0.01).结论 采用球囊扩张房间隔造口+肺动脉环缩的方法成功的建立了理想的肺血减少型先心病模型,该模型简单可靠、经济实用,十分接近临床的病理生理状态,为该类疾病的临床研究奠定了坚实的实验基础.

Abstract：

Objective To establish an animal model of congenital heart defect with decreased pulmonary blood flow for better understanding the pathophysiology of pulmonary vascular development and related regulatory mechanisms of congenital heart defect with decreased pulmonary blood flow. Method One to two months old pigs were randomly divided into three groups: control group(group C, n = 6)with right chest small incisions induced transient pulmonary blood reduction;light-moderate stenosis groups (group T1, n = 7):artificial atrial septum defect(ASD)plus controlled pulmonary artery banding to generate a systolic pressure gradient of 20-30 mm Hg(1 mm Hg = 0. 133 kPa);severe stenosis groups (group T2, n = 7): similar surgical procedures as group T1, and controlled pulmonary artery banding to generate a systolic pressure gradient ≥ 30 ～50 mm Hg. 64-slice computed tomography scanning was performed at one month post operation. Arterial blood gas analysis, hemoglobin value, pulmonary vessel,ASD and banding ring diameters and trans-pulmonary artery banding pressure(Trans-PABP)were determined at two months post operation. Results One pig died due to tracheal intubation accident in the C group, one pig died due to bowel obstruction in the T, group and two pigs died due to acute right heart failure and chronic heart failure respectively in T2 group. 64-slice CT angiography results showed that aortic diameter of T1 group was significantly lower than that of C group and banding diameter was significantly lower than aortic diameter in the T1 and T2 groups at one month post operation. Two months after operation, the size of ASD were(8.0 ± 0. 5)mm and(8.9 ± 1.4)mm(P ＞ 0. 05)respectively in the T1 and T2 groups after operation. The Trans-PABP was significantly higher in the T1 and T2 groups than in C group(P ＜0. 01), and the Trans-PABP was significantly higher in the T2 group than in T1 group(P ＜0. 01). PaO2 and SaO2 in the T1 and T2 groups were significantly lower than those in C group. Conclusion Artificial atrial septum defect combined pulmonary artery banding procedures could be successfully used to establish model of congenital heart defect with decreased pulmonary blood flow and this model could help to understand the pathophysiology and monitor therapy efficacy for patients with congenital heart defect with decreased pulmonary blood flow.     

259例室间隔缺损合并动脉导管未闭的外科治疗

目的:回顾性分析259例室间隔缺损(VSD)合并动脉导管未闭(PDA)外科治疗的临床资料,探讨其外科治疗经验和浅低温心脏不停跳技术的应用。方法:采用浅低温体外循环心脏不停跳技术行动脉导管结扎或缝合术以及VSD修补术,其中男性121例,女性138例,年龄2个月~38岁;合并畸形有房间隔缺损或卵圆孔未闭;主动脉缩窄;主动脉瓣下狭窄;瓦氏窦破裂;二尖瓣重度关闭不全等;直接分离结扎导管89例;其余病例经肺动脉切口缝合;其中利用补片修补动脉导管3例;合并畸形均同期手术矫治。结果:全组早期5例死亡,病死率1.9%,1例死于顽固心律失常,2例死于低心排出量综合征,2例死于手术后肺动脉高压危象合并肺部感染,随访3个月~7年,复查心脏超声均未发现VSD残余分流,无远期死亡病例。结论:VSD合并动脉导管未闭一旦明确诊断,应该早期手术治疗,以避免肺动脉高压的发生,手术中探查和进行肺动脉压力/主动脉压力比值测定有助于手术方式的选择和制定围手术期处理方案,心脏不停跳技术可作为安全有效的方法应用于VSD合并PDA的外科治疗。     

83例6个月以下婴儿室间隔缺损的外科治疗

目的 总结外科手术治疗小婴儿期室间隔缺损(VSD)的临床经验.方法 对2000-2005年83例＜6月龄VSD患儿,年龄12 d至6(4.3±1.2)个月,体重3～6.5(5.1±0.8)kg.膜周部VSD 74例,干下型VSD 8例,膜周部并肌部VSD1例.术前中、重度肺动脉高压69例(83.1%).所有患儿均在浅低温体外循环下手术.结果 全组手术死亡4例,手术病死率为4.8%.术后主要并发症为肺动脉高压危象、肺部感染和心律失常.结论 随着麻醉、体外循环、手术技术和围术期监护处理措施的进步,在小婴儿期施行VSD的外科手术已成为安全的治疗方式.术后对肺高压的处理是手术成功的关键.     

Imaging of recurrent ventricular septal defect and supravalvular pulmonary stenosis eight years after assumed total surgical repair of tetralogy of Fallot

A 42-year-old man underwent assumed total surgical repair of an acyanotic tetralogy of Fallot: a perimembranous ventricular septal defect (VSD) was closed with a dacron patch and myectomy of the infundibulum of the right ventricular outflow tract was performed. Reexamination eight years later revealed a large recurrent VSD and by surprise a narrow supravalvular ridge above rudimentary pulmonary cusps in the pulmonary trunk, leading to a pressure drop of 70 mmHg across the supravalvular stenosis. The latter finding was not recognized during the operation eight years before. The diagnosis could noninvasively be established by means of magnetic resonance imaging. During revision surgery the VSD was closed and an aortic homograft was inserted as conduit between the right ventricle and the pulmonary artery.     

Morphological characterisation of ventricular septal defects associated with coarctation of aorta by cross-sectional echocardiography.

Cross-sectional echocardiograms of 18 neonates and infants with coarctation of the aorta and ventricular septal defect were retrospectively assessed. With a combination of subcostal and precordial cuts the site and anatomical relations of the defects were determined. In one case there was a muscular trabecular ventricular septal defect. Three had a malalignment defect with associated left ventricular outflow tract obstruction. In two there was a doubly committed subarterial defect with associated malalignment of the point of continuity between the aortic and pulmonary valves and the crest of the trabecular septum. Twelve cases had a perimembranous defect, with varying degrees of extension into the inlet, trabecular, or outlet septum. In this group 10 had associated aortic override, with varying degrees of left ventricular outflow tract narrowing. Abnormal insertion of the tricuspid valve was observed in 10 cases, such that it partly obscured the ventricular septal defect. Thus in most cases of coarctation with ventricular septal defect, the morphology of the ventricular septal defect and ventricular outflow tracts is such that left ventricular ejection is directed towards the pulmonary artery rather than the aorta.     

Initial results of primary device closure of large muscular ventricular septal defects in early infancy using perventricular access

Objectives: To report our experience with the use of the Amplatzer muscular ventricular septal defect (VSD) occluder, using direct right ventricle free wall puncture for primary closure of muscular VSDs in infants. Background: Young infants with heart failure due to large or multiple muscular VSDs often require intervention at a stage when percutaneous device closure is impractical due to delivery system limitations. There are considerable benefits to avoiding bypass in these infants. Methods: Patients with suitable muscular VSDs, considered too small for transvenous closure, underwent perventricular muscular VSD closure under transesophageal echo (TEE) guidance. Results: Eight infants underwent the procedure. The median age was 14 weeks (2–41) with median weight of 4 kg (3–6.6). Four patients had additional VSDs and one patient had previous repair of coarctation. One patient had dextrocardia and severe left ventricular impairment due to an associated cardiomyopathy. The median VSD size was 8 mm (range, 4–10 mm). A single device (6–12 mm) was deployed in each patient, the size chosen being 1–2 mm larger than the TEE measurement. Two patients had subsequent pulmonary artery banding, with absorbable bands, because of moderate residual shunting through additional VSDs. The latest echo study at a median of 7.2 weeks (0.5–66) post device implantation revealed no patients with a hemodynamically significant shunt. Conclusion: Perventricular muscular VSD closure can be safely and effectively undertaken in small infants with suitable muscular defects, and this strategy can avoid the need for bypass. © 2008 Wiley‐Liss, Inc.     

Successful surgical repair of aortic atresia associated with normal left ventricle

Surgical repair of aortic atresia with a large ventricular septal defect and a normally developed left ventricle was accomplished in a 14 month old infant. Palliative surgery at age 23 days consisted of bilateral banding of pulmonary artery branches and replacement of the ductus arteriosus with a Goretex conduit. Corrective surgery was accomplished by closure of the ventricular septal defect, insertion of a valved conduit between the apex of the left ventricle and the subdiaphragmatic aorta, removal of the pulmonary artery bands and division of the pulmonary-aortic conduit. The patient has a mild coarctation of the aorta and remains asymptomatic at 2 years of age.     

Anatomic correction of transposition of the great arteries associated with ventricular septal defect: midterm results in 50 patients

From May 1977 to August 1982 50 patients who were 1.5 to 44 months old underwent anatomic correction of transposition of the great arteries (TGA) and closure of ventricular septal defect (VSD) at our institution. Thirty-nine patients underwent preliminary pulmonary arterial banding. Hospital mortality was 32%: four patients died as a result of technical problems, seven as a result of associated lesions, three of pulmonary hypertension, and two of left ventricular failure. Three other patients died after the first postoperative month (one of mediastinitis, one at reoperation for a residual VSD, and one of pulmonary hypertension). All 31 survivors are in excellent clinical condition and are in sinus rhythm after a mean follow-up period of 31 +/- 14 months. Twenty-five patients were reinvestigated by echocardiography (M mode and two-dimensional) and/or catheterization. Parameters of left ventricular contractility were within normal limits, but systolic aortic diameter was larger than normal (p less than .01). Seven patients had stenosis of the right ventricular outflow tract and five of these required reoperation. The two persistent problems with the anatomic correction of TGA associated with VSD are a relatively high operative mortality and secondary right outflow tract stenosis. However, use of this procedure results in better left ventricular function and fewer arrhythmias than does use of atrial repair techniques and also results in the use of the anatomically left ventricle as the systemic ventricle.     

婴幼儿主动脉缩窄合并心内畸形的Ⅰ期手术纠治

目的:总结主动脉缩窄合并心内畸形的外科治疗经验。方法:回顾分析2007年1月至2011年6月我院收治的婴幼儿主动脉缩窄合并心内畸形Ⅰ期手术治疗的临床病例。共计42例,其中男性26例,女性16例。平均年龄(1.6±0.9)岁(6 d～3岁),平均体质量(7.2±2.5)kg(2.8～12kg)。主动脉缩窄合并心内畸形:37例合并室间隔缺损,5例合并房间隔缺损,7例同时合并主动脉瓣二瓣畸形,手术方法采用双切口I期纠治5例,正中切口I期纠治37例,其中锁骨下动脉翻转法(8例)、人工补片法(6例)、端端吻合法(15例)、端端吻合+补片法(13例)。结果:本组42例中,死亡1例,术后9 d死于肺部感染,病死率2.4%。术后均复查心脏彩超显示降主动脉血流通畅,无明显狭窄,3例提示降主动脉内仍存在20～40 mmHg(1 mmHg=0.133 kPa)压差,心脏彩超示吻合口处狭窄,血流速度增快。结论:婴幼儿主动脉缩窄合并心内畸形采用深低温选择性脑灌注方法经胸骨正中切口I期手术治疗可以达到满意疗效。彻底切除缩窄段及导管组织是手术成功的关键。