体外循环下小儿肌部室间隔缺损的镶嵌治疗

目的 探讨镶嵌模式(hybrid procedure)治疗小儿肌部室间隔缺损(Mvsd)的手术方法及临床应用.方法 2006年1月至2010年6月,在体外循环心内直视手术下采用手术及封堵相结合的镶嵌技术矫治小儿Mvsd 45例,其中男20例,女25例;年龄52天～12岁;体重3～32 kg.7例为单个Mvsd,38例为多发性VSD.同时合并大血管错位(D-TGA)1例、法洛四联症(TOF)2例、肺动脉狭窄(PS)3例、动脉导管未闭(PDA)6例、房间隔缺损(ASD)6例、主动脉缩窄1例.均在心脏停跳后直视下将导引钢丝经三尖瓣孔自心脏右室面穿过VSD至左室面,直视下置入导引器,然后送入封堵器,完成Mvsd封堵.多发性VSD 38例,予自体心包片修补膜周部等较大的VSD,心内其他畸形同期完成矫治.结果 42例置入单枚封堵器(直径4～10 mm)、3例置入双枚封堵器(直径4～7 mm).手术经过顺利,术前左室射血分数(EF)均在正常范围,术后1天小于8月龄组EF均值低于正常,大于8月龄组EF正常,两者差异有统计学意义.术后常规每天给予5 mg/kg肠溶阿司匹林3～6个月.术后随访超声检查示封堵器位置无偏移,无残余分流,无二尖瓣、主动脉瓣反流、Ⅲ度传导阻滞及新发心律失常等.术后因重症感染放弃治疗1例,无远期死亡病例.结论 体外循环下镶嵌技术治疗小儿 Mvsd明显降低了围手术期并发症及病死率,简化了手术过程,降低了手术风险,是一种安全、有效的方法.

Abstract：

Objective To summarize the technique and clinical experience of hybrid procedure under cardiopulmonary bypass (CPB) in children with muscular ventricular septal defect (mVSD). Methods From January 2006 to June 2010, 45 cases of mVSD underwent hybrid procedure with CPB. mVSDs were closed with devices under direct vision in 45 cases. Of them, there were 20 males and 25 females. They ranged from 52 days to 12 years [mean (2.05 ±2.48) year] in age and from 3 to 30 kg [(11.93 ±7.70)kg] in body weight. Preoperatively, most of children were highly susceptible to respiratory tract infections. The hybrid approach was used in all patients with CPB under the guidance of transesophageal echocardiography (TEE). The diameter of mVSDs ranged from 2 to 7 mm under TEE. Of 45 cases, 40 patients had increased rates of pulmonary blood flow. 29 patients had left axis deviation and 12 cases had sinus arrhythmia on electrocardiography (ECG). 19 had other congenital heart lesions, including transposition of great arteries in 1 case, tetralogy of Fallot in 2, pulmonary artery stenosis in 3, patent ductus arteriosus in 6, atrial septal defects in 6) and aorta coactation in 1. The quantity of VSDs were from 1 to 7 (single, in 7; two, in 24 case; three, in 8 case; four, in 5 case and seven, in lease. 37 patients were combined with pulmonary hypertension in our cohort. 38 patients with another large VSD and 19 with other congenital heart lesions were required surgical repair at sometime. Results The hybrid procedures were undertaken in all 45 cases of this cohort. All cases were successful and no deaths occurred during operation. A total of 48 devices were implanted in 45 patients, including single devices in 42 cases (device size ranged from 4 to 10 mm) and two devices in 3 cases (device size ranged from 4 -7 mm). The average time on CPB was (58.28 ±20.70) min , while aortic crossclamp time was(34. 94 ± 14.75) min. In addition, the time on mechanical ventilation postoperatively ranged from 2 hours to 6 days. Compared to the older children, 20 infante aged less than 8 monhad a significant difference in cardiac function in the early postoperative period. One infant was given up treatment because of serious infection. Anather cases recovered with the use of supportive treatment, such as using vasoactive agents, digoxin, inhaling nitric oxide, diuresis, and so on. The enteric-coated aspirin was given at dose of 5 mg ? kg -1. day -1 for a period of 3 to 6 months as usual postoperatively. All patients attended follow-up at 1 week, 1 month, 3 months, 6 months, 1 year and 2 years post-procedure. No major complications were encountered during this period. All cases were no instance of migration of any of the devices, residual shunt, aortic regurgitation, atrioventricular valve dysfunction, Ⅲo atrial-ventricular conduction block, new arrhythmia, and so on. There are no death in long-term follow-up. Conclusion Hybrid procedure is safe and effective for the closure of congenital heart defects in children.     

表达谱基因芯片筛选体外循环前后心肌细胞因子的变化

Objestive Systemic inflarmmation may be triggered by injury, hypothermia, ischemia-reperfusion and the contact of the blood with foreign body during cardiopulmonary bypass (CPB). To determine the application values of gene chip technique in the clinical practice and the study of cardiovascular stagery, as well as to provide clues to the study of inflammatory responess during CPB, microarry for gene expression profiles was used to identify the differences in the gene expression of myocardium between pre-and post- CPB. Methods Six adult patients who underwent CPB from March to May in 2003 were involved. Samples of right atrium were col- lected before and at immediate end of CPB. BD AtlasTM cDNA Expression Arrays was used to identify the differences in the gene ex- pression of cytokines. The results were compared with that of semi-quantative RT-PCR. Resellts The mean age of 6 patients (5 males and 1 female) was (32.67± 11.72) years. The baseline heart function was gradeⅡin 3 cases and grade Ⅲ in 3 other cases. The baseline left ventricular ejection fraction(LVEF)was (58.17±7.91)%. The mere duration was (91.67±43.88) minutes for CPB and was (58.67±43.46) minutes for aorta blocking. The minimum nasopharynx/rectal temperture was (29.37±1.90)℃/ (32.15±1.52)℃. Gene expression profiles of cytokines in the myocardium pre- and post-CPB were analysed successfully. The ex- pression of IL-6, IFN-γ,Wnt5a, TNFRSF1B, a member of tumor necrosis factor receptor superfamily, PIGF and MFNG in the myo- cardium were unpregulated after CPB. Conclusion Microarray technique is applicable in the study of cytokines changes dying CPB. cDNA microarray identified pleliminarily the differences in the gene expression between pre- and post-CPB. These genes may be in- valved in inflammation and other psthophysiological responses incuced by CPB. The myocardiym is probably one of the major sources of cytokines during CPB. Further study may be helpful in understanding the llngthe development of inflammation during CPB, and eventually, reducing the post-operative complications.     

表达谱基因芯片筛选体外循环前后心肌细胞因子的变化

Objestive Systemic inflarmmation may be triggered by injury, hypothermia, ischemia-reperfusion and the contact of the blood with foreign body during cardiopulmonary bypass (CPB). To determine the application values of gene chip technique in the clinical practice and the study of cardiovascular stagery, as well as to provide clues to the study of inflammatory responess during CPB, microarry for gene expression profiles was used to identify the differences in the gene expression of myocardium between pre-and post- CPB. Methods Six adult patients who underwent CPB from March to May in 2003 were involved. Samples of right atrium were col- lected before and at immediate end of CPB. BD AtlasTM cDNA Expression Arrays was used to identify the differences in the gene ex- pression of cytokines. The results were compared with that of semi-quantative RT-PCR. Resellts The mean age of 6 patients (5 males and 1 female) was (32.67± 11.72) years. The baseline heart function was gradeⅡin 3 cases and grade Ⅲ in 3 other cases. The baseline left ventricular ejection fraction(LVEF)was (58.17±7.91)%. The mere duration was (91.67±43.88) minutes for CPB and was (58.67±43.46) minutes for aorta blocking. The minimum nasopharynx/rectal temperture was (29.37±1.90)℃/ (32.15±1.52)℃. Gene expression profiles of cytokines in the myocardium pre- and post-CPB were analysed successfully. The ex- pression of IL-6, IFN-γ,Wnt5a, TNFRSF1B, a member of tumor necrosis factor receptor superfamily, PIGF and MFNG in the myo- cardium were unpregulated after CPB. Conclusion Microarray technique is applicable in the study of cytokines changes dying CPB. cDNA microarray identified pleliminarily the differences in the gene expression between pre- and post-CPB. These genes may be in- valved in inflammation and other psthophysiological responses incuced by CPB. The myocardiym is probably one of the major sources of cytokines during CPB. Further study may be helpful in understanding the llngthe development of inflammation during CPB, and eventually, reducing the post-operative complications.     

表达谱基因芯片筛选体外循环前后心肌细胞因子的变化

Objestive Systemic inflarmmation may be triggered by injury, hypothermia, ischemia-reperfusion and the contact of the blood with foreign body during cardiopulmonary bypass (CPB). To determine the application values of gene chip technique in the clinical practice and the study of cardiovascular stagery, as well as to provide clues to the study of inflammatory responess during CPB, microarry for gene expression profiles was used to identify the differences in the gene expression of myocardium between pre-and post- CPB. Methods Six adult patients who underwent CPB from March to May in 2003 were involved. Samples of right atrium were col- lected before and at immediate end of CPB. BD AtlasTM cDNA Expression Arrays was used to identify the differences in the gene ex- pression of cytokines. The results were compared with that of semi-quantative RT-PCR. Resellts The mean age of 6 patients (5 males and 1 female) was (32.67± 11.72) years. The baseline heart function was gradeⅡin 3 cases and grade Ⅲ in 3 other cases. The baseline left ventricular ejection fraction(LVEF)was (58.17±7.91)%. The mere duration was (91.67±43.88) minutes for CPB and was (58.67±43.46) minutes for aorta blocking. The minimum nasopharynx/rectal temperture was (29.37±1.90)℃/ (32.15±1.52)℃. Gene expression profiles of cytokines in the myocardium pre- and post-CPB were analysed successfully. The ex- pression of IL-6, IFN-γ,Wnt5a, TNFRSF1B, a member of tumor necrosis factor receptor superfamily, PIGF and MFNG in the myo- cardium were unpregulated after CPB. Conclusion Microarray technique is applicable in the study of cytokines changes dying CPB. cDNA microarray identified pleliminarily the differences in the gene expression between pre- and post-CPB. These genes may be in- valved in inflammation and other psthophysiological responses incuced by CPB. The myocardiym is probably one of the major sources of cytokines during CPB. Further study may be helpful in understanding the llngthe development of inflammation during CPB, and eventually, reducing the post-operative complications.     

表达谱基因芯片筛选体外循环前后心肌细胞因子的变化

Objestive Systemic inflarmmation may be triggered by injury, hypothermia, ischemia-reperfusion and the contact of the blood with foreign body during cardiopulmonary bypass (CPB). To determine the application values of gene chip technique in the clinical practice and the study of cardiovascular stagery, as well as to provide clues to the study of inflammatory responess during CPB, microarry for gene expression profiles was used to identify the differences in the gene expression of myocardium between pre-and post- CPB. Methods Six adult patients who underwent CPB from March to May in 2003 were involved. Samples of right atrium were col- lected before and at immediate end of CPB. BD AtlasTM cDNA Expression Arrays was used to identify the differences in the gene ex- pression of cytokines. The results were compared with that of semi-quantative RT-PCR. Resellts The mean age of 6 patients (5 males and 1 female) was (32.67± 11.72) years. The baseline heart function was gradeⅡin 3 cases and grade Ⅲ in 3 other cases. The baseline left ventricular ejection fraction(LVEF)was (58.17±7.91)%. The mere duration was (91.67±43.88) minutes for CPB and was (58.67±43.46) minutes for aorta blocking. The minimum nasopharynx/rectal temperture was (29.37±1.90)℃/ (32.15±1.52)℃. Gene expression profiles of cytokines in the myocardium pre- and post-CPB were analysed successfully. The ex- pression of IL-6, IFN-γ,Wnt5a, TNFRSF1B, a member of tumor necrosis factor receptor superfamily, PIGF and MFNG in the myo- cardium were unpregulated after CPB. Conclusion Microarray technique is applicable in the study of cytokines changes dying CPB. cDNA microarray identified pleliminarily the differences in the gene expression between pre- and post-CPB. These genes may be in- valved in inflammation and other psthophysiological responses incuced by CPB. The myocardiym is probably one of the major sources of cytokines during CPB. Further study may be helpful in understanding the llngthe development of inflammation during CPB, and eventually, reducing the post-operative complications.     

表达谱基因芯片筛选体外循环前后心肌细胞因子的变化

Objestive Systemic inflarmmation may be triggered by injury, hypothermia, ischemia-reperfusion and the contact of the blood with foreign body during cardiopulmonary bypass (CPB). To determine the application values of gene chip technique in the clinical practice and the study of cardiovascular stagery, as well as to provide clues to the study of inflammatory responess during CPB, microarry for gene expression profiles was used to identify the differences in the gene expression of myocardium between pre-and post- CPB. Methods Six adult patients who underwent CPB from March to May in 2003 were involved. Samples of right atrium were col- lected before and at immediate end of CPB. BD AtlasTM cDNA Expression Arrays was used to identify the differences in the gene ex- pression of cytokines. The results were compared with that of semi-quantative RT-PCR. Resellts The mean age of 6 patients (5 males and 1 female) was (32.67± 11.72) years. The baseline heart function was gradeⅡin 3 cases and grade Ⅲ in 3 other cases. The baseline left ventricular ejection fraction(LVEF)was (58.17±7.91)%. The mere duration was (91.67±43.88) minutes for CPB and was (58.67±43.46) minutes for aorta blocking. The minimum nasopharynx/rectal temperture was (29.37±1.90)℃/ (32.15±1.52)℃. Gene expression profiles of cytokines in the myocardium pre- and post-CPB were analysed successfully. The ex- pression of IL-6, IFN-γ,Wnt5a, TNFRSF1B, a member of tumor necrosis factor receptor superfamily, PIGF and MFNG in the myo- cardium were unpregulated after CPB. Conclusion Microarray technique is applicable in the study of cytokines changes dying CPB. cDNA microarray identified pleliminarily the differences in the gene expression between pre- and post-CPB. These genes may be in- valved in inflammation and other psthophysiological responses incuced by CPB. The myocardiym is probably one of the major sources of cytokines during CPB. Further study may be helpful in understanding the llngthe development of inflammation during CPB, and eventually, reducing the post-operative complications.     

表达谱基因芯片筛选体外循环前后心肌细胞因子的变化

Objestive Systemic inflarmmation may be triggered by injury, hypothermia, ischemia-reperfusion and the contact of the blood with foreign body during cardiopulmonary bypass (CPB). To determine the application values of gene chip technique in the clinical practice and the study of cardiovascular stagery, as well as to provide clues to the study of inflammatory responess during CPB, microarry for gene expression profiles was used to identify the differences in the gene expression of myocardium between pre-and post- CPB. Methods Six adult patients who underwent CPB from March to May in 2003 were involved. Samples of right atrium were col- lected before and at immediate end of CPB. BD AtlasTM cDNA Expression Arrays was used to identify the differences in the gene ex- pression of cytokines. The results were compared with that of semi-quantative RT-PCR. Resellts The mean age of 6 patients (5 males and 1 female) was (32.67± 11.72) years. The baseline heart function was gradeⅡin 3 cases and grade Ⅲ in 3 other cases. The baseline left ventricular ejection fraction(LVEF)was (58.17±7.91)%. The mere duration was (91.67±43.88) minutes for CPB and was (58.67±43.46) minutes for aorta blocking. The minimum nasopharynx/rectal temperture was (29.37±1.90)℃/ (32.15±1.52)℃. Gene expression profiles of cytokines in the myocardium pre- and post-CPB were analysed successfully. The ex- pression of IL-6, IFN-γ,Wnt5a, TNFRSF1B, a member of tumor necrosis factor receptor superfamily, PIGF and MFNG in the myo- cardium were unpregulated after CPB. Conclusion Microarray technique is applicable in the study of cytokines changes dying CPB. cDNA microarray identified pleliminarily the differences in the gene expression between pre- and post-CPB. These genes may be in- valved in inflammation and other psthophysiological responses incuced by CPB. The myocardiym is probably one of the major sources of cytokines during CPB. Further study may be helpful in understanding the llngthe development of inflammation during CPB, and eventually, reducing the post-operative complications.     

超声心动图在外科微创封堵治疗室间隔缺损中的应用

目的 探讨超声心动图在外科微创封堵治疗室间隔缺损中的应用价值.方法 73例室间隔缺损病人,合并膜部瘤形成35例,其中21例膜部瘤合并多处破口.所有病例采用胸骨旁小切口,置入等边封堵器闭合VSD.术中TEE监测封堵过程,引导封堵器的放置,术后即刻评价疗效.术后1年采用经胸超声心动图(TTE)跟踪随访.结果 73例在超声心动图监测引导下置入封堵器均获成功,术中即刻用经食管超声检查显示,9例封堵器边缘仍存在微量至少量残余分流;术后48 h复查显示7例仍存在微量分流.术后1年随访中4例仍有微量残余分流,但显示左心室内径、容积较术前明显减小,基本恢复正常水平,肺动脉收缩压较术前明显减低.结论 超声心动图在外科微创治封堵疗的室间隔缺损中,对术前适应证的筛选、术中实时监测及术后疗效评定方面具有重要作用.

Abstract：

Objective To assess the application of echocardiography in mini-invasive surgical device closure of ventricular septal defect (VSD). Methods 73 VSD patients including 35 with aneurysm formation and among them 21 with multi-defects in the aneurysm were treated by mini-invasive device closure. A closure device was positioned to the defect through parastemal mini-incision in all patients. TEE was used to monitor the whole procedure, to guide the device positioning and to evaluate the curative effect instantly after operation. All patients were evaluated by TTE one year postoperatively. Results All patients were successfully positioned closure devices by TEE guiding. 9 cases were found with trace to small amount residual shunt instantly after operation. 7 cases still had small amount residual shunt at the time of 48 hours after the operation. In the one year follow-up, 4 cases had residual shunt, but the size and volume of left ventricle were significantly reduced than those before operation, and the pulmonary artery systolic pressure was also reduced. Conclusion Echocardiography possesses an important role in preoperative indication screening, intraoperation monitoring and evaluating the curative effect postoperatively.     

自体肺动脉补片主动脉弓成形治疗婴儿主动脉缩窄合并弓发育不良

Objective To discuss the operative techniques and results of coarctation resection plus aortoplasty with pulmonary autograft patch for coarctation of the aorta combined with hypoplastic aortic arch in infant.Methods Between May 2007 and Dec 2009,14 cases including 9 males and 5 females with caorctation of the aorta and hypoplastic aortic arch underwent coarctation resection plus aortoplasty with pulmonary autograft patch in our hospital.The age ranged from 23 days to 17 months,with a median of 4.33 months.The mean body weight was (6.14 ±2.36) kg.All patients were diagnosed as aortic coarctation combined with VSD and hypoplastic aortic arch.The surgery was performed under deep hypothermia cardiopulmonary bypass with selective cerebral perfusion in 8 cases and circulation arrest in 6 cases.Fresh pulmonary autograft patch harvested from the main pulmonary artery was used for aortoplasty.The associated VSD was repaired in the same stage.Results All patients survived except one died from circulatory failure during the perioperative period.Low cardiac output syndrome occurred in another case who was cured afterwards by correspondent treatments.No residual obstruction was detected by echocar-diography after the operation.Follow-up was carried out in 13 cases from 4 months to 3 years.Echocardiographic examination showed that the pressure gradient across the aortic arch was less than 16 mm Hg in all cases.The blood velocity at the descending aortic arch was not significantly changed during the follow-up period as compared with that of the immediate after operation.Computed tomography showed that the morphology of aortic arch was normal.The left bronchus compression was relieved obviously or totally disappeared in patients who suffered from left bronchus stenosis before operation,and no aortic aneurysm were detected in these patients.Conclusion Conclusion Coarctation resection plus aortoplasty with pulmonary autograft patch is the optimal surgical method for treating coarctation of the aorta combined with hypoplastic aortic arch in infant.     

自体肺动脉补片主动脉弓成形治疗婴儿主动脉缩窄合并弓发育不良

Objective To discuss the operative techniques and results of coarctation resection plus aortoplasty with pulmonary autograft patch for coarctation of the aorta combined with hypoplastic aortic arch in infant.Methods Between May 2007 and Dec 2009,14 cases including 9 males and 5 females with caorctation of the aorta and hypoplastic aortic arch underwent coarctation resection plus aortoplasty with pulmonary autograft patch in our hospital.The age ranged from 23 days to 17 months,with a median of 4.33 months.The mean body weight was (6.14 ±2.36) kg.All patients were diagnosed as aortic coarctation combined with VSD and hypoplastic aortic arch.The surgery was performed under deep hypothermia cardiopulmonary bypass with selective cerebral perfusion in 8 cases and circulation arrest in 6 cases.Fresh pulmonary autograft patch harvested from the main pulmonary artery was used for aortoplasty.The associated VSD was repaired in the same stage.Results All patients survived except one died from circulatory failure during the perioperative period.Low cardiac output syndrome occurred in another case who was cured afterwards by correspondent treatments.No residual obstruction was detected by echocar-diography after the operation.Follow-up was carried out in 13 cases from 4 months to 3 years.Echocardiographic examination showed that the pressure gradient across the aortic arch was less than 16 mm Hg in all cases.The blood velocity at the descending aortic arch was not significantly changed during the follow-up period as compared with that of the immediate after operation.Computed tomography showed that the morphology of aortic arch was normal.The left bronchus compression was relieved obviously or totally disappeared in patients who suffered from left bronchus stenosis before operation,and no aortic aneurysm were detected in these patients.Conclusion Conclusion Coarctation resection plus aortoplasty with pulmonary autograft patch is the optimal surgical method for treating coarctation of the aorta combined with hypoplastic aortic arch in infant.     

食道超声引导下小切口室间隔缺损封堵术的疗效分析

目的评估食道超声引导下小切口室间隔缺损(室缺)封堵术的疗效。方法 2008年10月～2009年6月,45例先天性室间隔缺损患儿,男27例,女18例,平均年龄3.5岁(14个月～11岁),平均体重16.8 kg(9～30 kg)。经胸心脏超声显示室间隔缺损,8例为嵴内型,36例为膜部型(其中6例伴有膜部瘤形成),1例为肌部型。缺损直径3～10 mm,平均4.5mm。气管插管全身麻醉后,经胸骨下段正中切口,长3～5 cm。在食道超声的引导下,经右室游离壁置入腰部直径5～16 mm的国产室缺封堵器。结果 45例均成功封堵,无中转体外循环病例。42例术后无残余分流,3例封堵后即刻超声示少量残余分流,术后5天复查超声示分流消失。2例术后心电图示右束支传导阻滞,无Ⅲ度房室传导阻滞发生,余43例术后心电图无异常改变。术后住院时间3～7 d,平均4.4 d。随诊3～6个月,1例术后1个月大量心包积液,经心包穿刺后痊愈,余44例均恢复良好,无Ⅲ度房室传导阻滞,无残余分流,无封堵器晚期移位,无瓣膜关闭不全加重。结论食道超声引导下小切口室缺封堵术创伤小,安全性高,近期疗效满意。     

经食管超声在室间隔缺损小切口封堵术中的应用

目的评价TEE在室间隔缺损（VSD）外科小切口封堵术中的价值。方法 25例VSD患者接受外科小切口封堵术,其中膜周型缺损19例,嵴内型缺损6例;缺损直径3～9mm,平均（5.25±3.47）mm。所有患者术前均接受TTE检查评估VSD。气管插管全身麻醉后,在TEE引导下,经右心室游离壁置入VSD封堵器,并于术后即刻评价手术效果。结果 25例均封堵成功,19例使用等边封堵器,6例使用偏心封堵器。25例术后即刻超声及术后超声观察,室间隔水平均无分流信号。结论 TEE在VSD小切口封堵术术中监测及术后评价中具有重要价值。     

Repair of ventricular septal defect in adults

To evaluate the outcome of ventricular septal defect (VSD) with long duration of haemodynamic derangement, a retrospective study was made of 42 consecutive patients who underwent closure of VSD as adults (age range 15-48, mean 27 years). The mean systolic pulmonary arterial pressure was 53 mmHg, mean pulmonary vascular resistance 2.5 Wood units and mean pulmonary/systemic flow ratio 2.4. VSD was complicated by aortic regurgitation in 12 cases, mitral regurgitation in 4, and sinus of Valsalva fistula in 6 cases. There were 15 supracristal, 24 infracristal and 3 muscular VSDs. In addition to VSD closure, surgery included aortic valve replacement (7 cases), mitral valve replacement (2), valve repair by suture (7) and repair of Valsalva sinus fistula (6 cases). Two patients died in the early postoperative period and two during follow-up (1-10, mean 4.5 years). The early and the late mortality were related to large infracristal VSD, pulmonary hypertension and irreversible pulmonary vascular changes which could not be anticipated on the basis of high calculated shunt flow at preoperative catheterization. No patient with supracristal VSD died. Recurrent VSD was diagnosed in five patients, three of whom needed reoperation and recovered uneventfully. Reduction of heart size and improved exercise tolerance were the most pertinent follow-up findings. The results suggest that large supracristal VSD with aortic valve involvement can be successfully closed in adults, but that the prospect for large infracristal VSD is less favourable if correction is postponed until adulthood.     

经心室穿刺封堵婴幼儿非肌部室间隔缺损39例

目的 探讨经心室穿刺封堵婴幼儿非肌部室间隔缺损(VSD)的临床效果.方法 2007年4月至2008年2月,治疗39例非肌部VSD病儿,男16例,女23例.年龄12～36个月,平均(14.5±7.8)个月;体重8.5～18.0 kg,平均(12.4±2.3)kg.其中膜周部VSD 34例、干下型3例、嵴内型2例.缺损直径3.0～11.0mm,平均(6.1±2.0)mm.均接受非体外循环下经右心室穿刺封堵VSD治疗.结果 全组均无需输血.37例封堵成功,2例因发生中度主动脉瓣反流改行直视手术.置入封堵器直径4～12 mm,平均(8.2±2.0)mm.术后膜周部VSD者中三尖瓣反流程度减轻3例,新出现微量至轻度三尖瓣反流8例;6例出现不完全性右柬支传导阻滞.术后住院3～5 d,平均(3.4±0.4)d.结论 经心室穿刺行VSD封堵术对婴幼儿非肌部VSD而言是一种有效和安全的治疗手段.     

Hybrid procedure with cardiopulmonary bypass for muscular ventricular septal defects in children

Objective: To summarize the technique and clinical experience of the hybrid procedure with cardiopulmonary bypass in children with muscular ventricular septal defect (mVSD). Methods: From January 2006 to June 2010, 45 cases of mVSD underwent hybrid procedures with cardiopulmonary bypass (CPB) under the guidance of transesophageal echocardiography. mVSDs were closed with devices under direct vision in the 45 cases. Fourteen patients had another lesion that required surgical repair. Large membranous VSDs were closed with a pericardial patch after the initiation of CPB in 38 cases. Results: Out of the 45 cases, 42 had only one occluder and three had two occluders. The size of the device for mVSD closure ranged from 3 to 8 mm. All cases recovered smoothly after treatment without residual shunting, aortic or mitral valve regurgitation, or restriction of surrounding structures. All the children survived the operation with no late deaths during the follow-up. Conclusion: The hybrid procedure is safe and effective for the closure of congenital heart defects in children.     

Surgical management of muscular trabecular ventricular septal defects

Despite recent advances in diagnosis, surgical techniques, and postoperative care of children with congenital cardiac defects, muscular trabecular ventricular septal defects (VSDs) are still a therapeutic challenge. Among these defects, it is more difficult to achieve secure and complete closure of low trabecular or apical VSDs because of the presence of numerous muscular trabeculations overlying the defect. When they are associated with “Swiss cheese”-type of VSDs, it is almost impossible to visualize the true edges of the defect through the transatrial approach. Consequently, there remains an unacceptable incidence of mortality and morbidity when compared to those that occur with closure of the usual perimembranous VSD. Although various techniques for closure of these difficult trabecular VSDs have been attempted, there is still a significant incidence of complications in the surgical management of trabecular VSDs, mostly significant residual shunts, a need for multiple reoperations, and severe left ventricular dysfunction. This article describes the anatomical details and classification of muscular trabecular VSDs. It also reviews several techniques currently utilized and their outcomes.     

The optimal procedure for the great arteries and left ventricular outflow tract obstruction. An anatomical study.

OBJECTIVE: To describe the optimal surgical strategy in heart specimens with transposition of the great arteries (TGA) and left ventricular outflow tract obstruction (LVOTO). METHODS: Thirty-three specimens with LVOTO were selected: TGA with intact ventricular septum (TGA/IVS) (10), TGA/VSD (21), and Taussig-Bing (2). RESULTS: LVOTO in TGA/IVS consisted of combinations of bicuspid pulmonary valve (four), subpulmonary fibrous ridge (four), obstructive muscular conus (two) and bulging muscular septum (four). Arterial switch operation (ASO) with LVOTO resection/valvotomy was feasible in nine hearts. Obstructive anterior papillary muscle prohibited LVOTO relief in one specimen. In TGA/VSD and Taussig-Bing LVOTO consisted of combinations of bicuspid (nine) or unicommissural (one) pulmonary valve, fibrous ridge (three), obstructive muscular conus (five), malaligned outlet septum (six), accessory mitral valve tissue (two), straddling mitral valve (two) and anterior mitral valve rotation (four). VSDs were subpulmonary in 13 (9 perimembranous, 4 muscular), subaortic in 3 (2 perimembranous, 1 anterior muscular), doubly committed in 2, inlet in 3 (2 perimembranous, 1 muscular), non-committed and anterior in 1, and finally 1 VSD extended both into inlet and subpulmonary outlet septum. LVOTO resection and ASO with VSD closure was possible in 10. In six specimens, both a Rastelli and a Nikaidoh operation were feasible. For two hearts, a Nikaidoh procedure was the only option, while Rastelli was considered optimal in another specimen. Mitral valve anomalies prevented LVOTO relief in four, only permitting for Senning/VSD closure (one) or univentricular palliation (three). CONCLUSIONS: LVOTO resection and pulmonary valvotomy frequently permits an ASO. Inlet VSD, impossibility of VSD enlargement, straddling mitral valve, distant aorta and small right ventricle make the Nikaidoh procedure the best option. Mitral anomalies preventing LVOTO relief can make biventricular repair impossible.     

经食管超声心动描记术在先天性心脏病镶嵌治疗中的应用

目的观察TEE在非体外循环下先天性心脏病镶嵌治疗中的临床应用价值。方法回顾性分析167例TTE初筛拟行先天性心脏病镶嵌治疗的患者,术前TEE检查进一步筛选患者,指导选择适当封堵器,术中TEE监测引导置入封堵器并评价其疗效。结果本组167例中,24例房间隔缺损(ASD)和121例室间隔缺损(VSD)成功完成镶嵌治疗,1例ASD和5例VSD术后即刻TEE可见一丝残余分流,术后3个月复查1例ASD和4例VSD残余分流消失,1例VSD右心室流出道血流速度稍加快,1例VSD术前无主动脉瓣反流,术后出现轻-中度反流。3例ASD和9例VSD镶嵌治疗不顺利,转体外循环。对1例ASD和2例VSD由于缺口过小而放弃治疗。7例VSD合并主动脉瓣脱垂直接放弃镶嵌治疗,转修补术。结论在非体外循环下先天性心脏病的镶嵌治疗中,TEE能为选择适应证和封堵器提供技术支持,实时监测指导及评价手术,降低手术风险,提高手术成功率。