超声心动图对Gore-Tex片作单瓣重建右心室流出道的早期疗效研究

目的：应用超声心动图评价0.1mm厚的Gore-Tex片作肺动脉单瓣重建右心室流出道（RVOT）的早期临床效果。方法：我院2002年6月~2010年3月应用超声心动图对75例合并肺动脉狭窄或闭锁的先天性心脏病患者采用0.1mm厚的膨体聚四氟乙烯（Gore-Tex）片作肺动脉单瓣重建ROTV的临床研究。其中包括57例法洛四联症合并肺动脉狭窄,10例I型肺动脉闭锁,4例肺动脉瓣缺如,2例右心室双出口合并肺动脉狭窄,永存动脉干及完全型大动脉转位、室间隔缺损、肺动脉狭窄各1例。应用飞利浦公司经胸IE33彩色多普勒超声心动图仪（TTE）,探头频率为2.5~3.75MHz。选择TTE切面：胸骨旁左室长轴切面、大动脉短轴切面、左室流出道、肺动脉主干及分叉处短轴切面、心尖四腔心切面、剑突下右室流出道、流入道及左心室流入道长轴切面,术后1个月内复查,左、右心室收缩功能,观察Gore-Tex片状态,有无单瓣的狭窄、关闭不全及术后并发症。结果：应用TTE术后检查75例,其中62例（82.6%）Gore-Tex片作肺动脉单瓣重建右心室流出道（RVOT）早期瓣膜活动功能正常,13例（17.33%）单瓣固定在开放状态;72例（97.3%）均无肺动脉单瓣狭窄,3例（3%）单瓣轻度狭窄;21例（28%）肺动脉瓣轻度返流（PI）,5例（6.7%）中度返流;7例（8%）心包积液,其中6例少量,1例大量;4例残余室间隔缺损,直径均为1.5~2mm,其中3例围膜部,1例肌部。左室射血分数（LVEF）0.53~0.85,右室射血分数（RVEF）0.52~0.71,左室舒张末期容积指数0.28~0.62ml/m2。     

法乐四联症根治术后残余肺动脉瓣狭窄的介入治疗及疗效评价

目的对法乐四联症根治术后残余肺动脉瓣狭窄进行经皮球囊成形术并评价其疗效。方法对16例患者行经皮肺动脉瓣球囊成形术,年龄1~23岁,平均(7.8±6.3)岁,术前超声心动图测量肺动脉瓣跨瓣峰值收缩压差为(81.83±23.17)mmHg(55~130mmHg)。术后24小时、6个月及1年行超声心动图、X线平片及心电图进行随访。结果 14例使用聚乙烯球囊导管进行扩张,2例使用Inoue球囊导管,球囊平均直径(19.58±4.03)mm;扩张后肺动脉-右心室收缩压差由(80.50±27.49)mmHg降至(46.25±29.19)mm(P0.01),4例(25.0%)肺动脉-右心室收缩压差仍大于50mmHg。随访中肺动脉瓣跨瓣峰值压差与术后早期差异无统计学意义。结论法乐四联症术后75.0%残余肺动脉瓣狭窄的患者可以通过介入治疗有效降低肺动脉-右心室收缩压差,从而避免二次手术。     

液氮深低温保存同种带瓣管道移植治疗复杂先天性心脏病17例

目的:同种带瓣管道已经广泛应用于复杂先天性心脏病的治疗,临床应用中显示了良好的近中期效果,但其长期耐久性还有待提高.回顾性分析自行制备和液氮深低温保存的同种带瓣管道治疗复杂先天性心脏病的临床应用效果.方法:①选择南京人学医学院附属鼓楼医院心胸外科2003-01/2007-10应用同种带瓣管道治疗复杂先天性心脏病的患者17例,其中法洛四联症7例:肺动脉闭锁合并室问隔缺损3例:先天性主动脉瓣狭窄2例:右心室双出口合并室间隔缺损和肺动脉狭窄2例;大动脉转位合并室间隔缺损和肺动脉狭窄3例,其中1例合并右心室双出口.②手术方式:肺动脉带单瓣血管片作右心室流出道跨环补片7例,Rastelli术4例,肺动脉-右心室流出道外管道连接术3例,自体肺动脉瓣移植术2例,全腔静脉肺动脉连接术1例.⑨观察术后早期及随访期间患者的治疗效果.结果:①16例患者手术顺利完成,1例术中心脏复跳后不能脱离体外循环死亡.术后死亡1例,死亡原因为低心排综合征、心力衰竭:其余15例均痊愈出院.②随访1～48个月,15例患者生活质量良好.超声心动图示植入的同种带瓣管道无明显钙化、衰败.结论:液氮深低温保存同种带瓣管道治疗复杂先天性心脏病早、中期效果良好,远期防止同种带瓣管道钙化、衰败是临床进一步的研究重点.     

法洛四联症的外科治疗

法洛四联症(TOF)是最常见的紫绀属先天性心脏病,占先天性心脏病的10%,含有四种心血管畸形:(1)漏斗部及肺动脉狭窄,漏斗部狭窄随年龄增长逐渐加重;(2)室间隔缺损;(3)主动脉骑跨;(4)右心室肥厚。TOF病理改变范围大,主要表现为肺动脉狭窄程度轻重不一,从轻度狭窄到完全闭锁;患者缺氧程度不一,动脉血氧饱和度从正常到低于70%;对患者的影响不一,重者在新生儿期即因缺氧危及生命,少数轻者可存活至70多岁。1945年Blalock等[1]成功地进行了第一例TOF的姑息手术——体肺动脉分流术,1954年Lillehei等[2]采用交叉循环方法成功地施行了TOF的心内修复术,1955年Kirkli     

重症法洛四联征应用Gore-Tex人造血管行体-肺分流术临床观察

目的:探讨重症法洛四联征应用Gore-Tex人造血管行体-肺中心分流术的临床经验,评价其手术疗效。方法:21例重症法洛四联征患者应用Gore-Tex人造血管行升主动脉至主肺动脉的分流手术。19例在非体外循环下手术,2例在体外循环下手术。Gore-Tex人造血管直径4~10 mm。结果:手术死亡2例,病死率9.5%(2/21)。全组均获得理想的血流量,术后经皮血氧饱和度、动脉血氧分压均较术前增加,差异有统计学意义(P<0.01);主肺动脉直径和左、右肺动脉直径较术前增大,差异有统计学意义(P<0.05);左室舒张末期容积指数较术前增加,差异有统计学意义(P<0.05)。17例(80.95%)患者在术后1年内接收二期根治手术,无死亡病例。其余2例患者缺氧症状,左心室及肺动脉发育均明显改善,等待根治手术。结论:应用合适口径Gore-Tex人造血管行体-肺分流术可明显改善重症法洛四联征症状、体征,为重症法洛四联征根治术创造良好的条件。     

应用PTFE人工单叶肺动脉瓣实施右心室流出道重建术的护理配合

目的总结应用PTFE人工单叶肺动脉瓣实施右心室流出道重建矫治先天性心脏病的手术配合。方法用经修剪的PTFE片缝制人工单叶肺动脉瓣及自体心包片行右心室及主肺动脉补片扩大。结果21例中死亡1例,病死率4.76％,余20例术后恢复良好,除第1例患儿存在中度肺动脉返流外,其余患者均无返流;全部患儿均无肺动脉或右心室流出道梗阻。结论在行跨肺动脉瓣右心室流出道补片扩大术中,应用PTFE人工单叶肺动脉瓣可以有效地避免术后肺动脉瓣返流,改善右心室功能。     

肺动脉右心室流出道单瓣重建术后并发症的观察及护理

复杂先天性心脏病往往合并重度肺动脉瓣狭窄、发育不良或闭锁,已往外科修复采用跨瓣环补片加宽右心室流出道(right ventricular outflow tract,RVOT),但解除RVOT狭窄的同时往往会导致肺动脉瓣反流,而长期肺动脉瓣反流可致右心室容量及压力负荷增加,最终导致心律失常、运动耐力下降或心力衰竭[1-2]。我科于2002年6月至2011年11月对例合并肺动脉狭窄或闭锁的先天性心脏病     

手术治疗先天性主动脉瓣上狭窄42例疗效观察

目的探讨先天性主动脉瓣上狭窄(supravalvular aortic stenosis, SVAS)患儿行手术治疗的安全性及近中期疗效。方法先天性SVAS患儿42例,36例在体外循环下行主动脉瓣窦-升主动脉补片扩大成形术,6例在体外循环下行升主动脉-主动脉弓延长补片成形术。记录体外循环时间、主动脉阻断时间、呼吸机辅助时间、术后ICU停留时间及住院时间;术后7 d复查心脏CT造影,检测主动脉瓣上及合并病变是否有残余梗阻;分别于术前及术后7 d行超声心动图检查,测定主动脉瓣上流速、主动脉跨瓣压差及左室射血分数;随访观察近中期预后。结果 42例均顺利完成手术,体外循环时间(64.7±24.6)min,主动脉阻断时间(51.8±22.3)min,呼吸机辅助时间(25.8±26.4)h,术后ICU停留时间(12.3±6.2)d,住院时间(16.3±6.2)d;术后7 d心脏CT造影示主动脉瓣上及合并病变无残余梗阻;术后7 d主动脉跨瓣处压差[(20.34±8.56)mm Hg]较术前[(67.46±26.16)mm Hg]降低(P0.05),主动脉瓣上流速[(1.63±0.68)m/s]较术前[(4.13±1.04)m/s]减慢(P0.05),左室射血分数[(67.14±6.03)%]较术前[(61.35±5.12)%]增高(P0.05);住院期间死亡2例,余患儿均顺利出院;随访至1个月～5年6个月,失访4例,余患儿心脏CT造影示均无残余梗阻。结论先天性SVAS患儿行手术治疗可解除主动脉瓣上及合并病变梗阻,改善心功能,安全性高,近中期效果满意。     

彩色多普勒超声心动图在法洛三联症介入治疗中的价值

目的 探讨彩色多普勒超声心动图(CDE)在法洛三联症(TOF)介入治疗中的价值.方法 选取31例经CDE筛选的31例TOF患者进行介入治疗,通过CDE术中监测及术后1、3、6个月跟踪随访,判断介入治疗的疗效.结果 根据CDE图像特征表现对28例做出准确诊断,诊断符合率90.3％,漏诊3例,其中2例被误诊为继发孔房间隔缺损(ASD),1例被误诊为室间隔完整的肺动脉闭锁.二维超声心动图测量ASD直径5～28 mm,平均(17.3±10.2) mm,与介入治疗术中测量ASD直径7～30 mm,(18.4±11.1) mm]比较,差异无统计学意义;连续多普勒估测跨肺动脉瓣压力阶差36～154 mm Hg(1 mm Hg=0.133 kPa),平均(92±54) mm Hg,与心导管测量跨肺动脉瓣压力阶差[30～140 mm Hg,平均(85±48) mm Hg]比较,差异无统计学意义;介入治疗后连续多普勒估测跨肺动脉瓣压力阶差下降至16～30 mm Hg,平均(23±6) mm Hg.31例入选患者经实施介入治疗后均获得成功.介入治疗后1、3、6个月随访,无过房间隔分流束血流信号.结论 CDE在TOF介入治疗术前筛选适应证,判断ASD大小、位置、残余边缘和肺动脉瓣狭窄程度,术中监测和术后随访判断疗效,判定封堵器位置是否正常、有无过房间隔残余分流束血流信号及估测跨肺动脉瓣压力阶差下降程度中具有重要价值.     

改良Blalock-Taussig分流术治疗法洛四联症25例

目的:探讨改良Blalock-Taussig分流术治疗法洛四联症的优点。方法:行改良Blalock-Taussig术治疗法洛四联症患儿25例,术后随访3~15个月,观察疗效。结果:随访3~15个月,术后出现肺不张1例;胸腔积液2例;人工血管栓塞1例,于术后1个月再行对侧改良Blalock-Taussig术;人工血管狭窄3例;余21例人工血管通畅,患儿血氧饱和度由术前70%~83%上升到83%~91%,差异有统计学意义(P<0.05)。术后6~15个月行2期根治术15例,因心力衰竭死亡1例,14例疗效满意。结论:改良Blalock-Taussig分流术可以改善法洛四联症患儿缺氧情况,促进肺动脉发育,为2期手术提供机会,降低病死率。     

产前超声诊断胎儿法洛四联症的价值及体会

目的探讨胎儿超声心动图对单纯及复杂法洛四联症(TOF)的产前诊断价值。方法回顾性分析23例经超声心动图诊断为胎儿TOF的声像图表现及随访资料。结果单纯TOF 19例,表现为室间隔缺损、肺动脉狭窄和主动脉骑跨。复杂TOF 4例,包括:①TOF合并肺动脉闭锁2例:表现为右室流出道为盲端,肺动脉细窄、不易显示,肺动脉及动脉导管内逆向血流;②TOF合并肺动脉瓣缺如2例:表现为肺动脉主干、分支瘤样扩张,肺动脉瓣反流等。结论超声心动图产前诊断胎儿TOF及其分型具有较大的临床价值,并可动态观察其右室流出道梗阻、肺动脉及其瓣膜的发育及进展情况。     

Surgical management of pulmonary atresia, and critical pulmonary stenosis with intact ventricular septum

Our experience with the surgical management of hypoplastic right ventricle with intact ventricular septum has included 20 patients with pulmonary atresia and 3 patients with critical pulmonary stenosis. Group 1 consisted of 7 infants who were treated by transarterial pulmonary valvotomy. Four of them were lost shortly after surgery. Group 2 included 11 neonates and one infant who were treated by systemic-pulmonary shunt with 2 operative death. Group 3 included 4 infants who underwent primary prosthetic enlargement of the right ventricular outflow tract. Two of the 4 were lost in the early postoperative period. Eight of 13 patients in groups 1 and 2 who survived the initial palliative procedures underwent additional operations to enlarge the right ventricular outflow tract, and 5 patients survived. However, adequate right ventricular and tricuspid valve growth was obtained in only patients in whom the prosthetic enlargement of the right ventricle was performed in early infancy. These findings strongly suggest the necessity to decompress the right ventricle completely, as early as possible. In conclusion, 1) transarterial pulmonary valvotomy seldom provided satisfactory decompression of the right ventricle, and operative risk was high. 2) systemic-pulmonary shunt to re-establish pulmonary blood flow improved neonatal survival, although this procedure may not provide a satisfactory long term palliation. 3) primary or secondary operative enlargement of the right ventricle in early infancy provided decompression of the right ventricle and accelerated substantial right ventricular growth. For patients without an infundibulum or for those in whom attempts to induce tricuspid valve growth had failed, a Fontan type procedure seemed to be the procedure of choice.     

先天性右心梗阻性疾病的彩色多普勒超声诊断

本组18例,其中17例经手术证实的先天性右心梗阻性疾病患者,包括肺动脉分支狭窄、肺动脉瓣膜性狭窄,右室漏斗部狭窄、右室双腔心.通过彩色多普勒超声与手术对照研究,表明彩色多普勒超声能准确地显示梗阻的部位,程度,合并畸形,及血流动力学改变,对本病的诊断具有决定性的价值.     

Variations in right ventricular outflow tract morphology following repair of congenital heart disease: implications for percutaneous pulmonary valve implantation.

OBJECTIVE: Our aim was to identify sub-groups of right ventricular outflow tract morphology that would be suitable for percutaneous pulmonary valve implantation and to document their prevalence in our patient population. MATERIALS AND METHODS: Eighty-three consecutive patients with right ventricular outflow tract dysfunction (5-41 years, 76% tetralogy of Fallot) referred to our center for cardiovascular magnetic resonance were studied. A morphological classification was created according to visual assessment of three-dimensional reconstructions and detailed measurement. Diagnosis, right ventricular outflow tract type, surgical history and treatment outcomes were documented. RESULTS: Right ventricular outflow tract morphology was heterogeneous; nevertheless, 5 patterns were visually identified. Type I, a pyramidal morphology, was most prevalent (49%) and related to the presence of a transannular patch. Other types (II-V) were seen more commonly in patients with conduits. Two patients had unclassifiable morphology. Ninety-five percent of patients were assigned to the correct morphological classification by visual assessment alone. Percutaneous pulmonary valve implantation was performed successfully in 10 patients with Type II-V morphology and in 1 patient with unclassifiable morphology. Percutaneous implantation was not performed in patients with Type I morphology. Only right ventricular outflow tract diameters < 22 mm in diameter were suitable for the current device. CONCLUSIONS: We have created a morphological classification of the RVOT in patients referred for assessment of RVOT dysfunction. Though only 13% of our patients underwent percutaneous implantation, > 50% of outflow tract morphologies may be suitable for this approach, in particular with the development of new devices appropriate for larger outflow.     

Prenatal sonographic diagnosis of tetralogy of fallot

The objective of this report was to emphasize the sonographic findings of tetralogy of Fallot (TOF) during the prenatal period. Four fetuses with TOF were prenatally diagnosed at gestational ages of 25, 28, 25, and 32 weeks. Based on this small series, prenatal sonographic findings suggestive of TOF may be summarized as follows: (1) a large aortic root, which is the most common prenatal sonographic finding, (2) a small pulmonary artery or stenosis of the right ventricular outflow tract, (3) a ventricular septal defect in the outlet portion of the septum, (4) an overriding aorta, which is best seen in the long-axis view, and (5) right ventricular hypertrophy. Other findings such as hydrops fetalis or polyhydramnios may also be helpful especially in the case of dysplastic pulmonary valves. The demonstration of a normal aortic root would render the presence of TOF unlikely. TOF with pulmonary atresia can be confused with truncus arteriosus, in which both pulmonary arteries arise from the ascending aorta. How-ever, with careful examination along the course of the ascending aorta, there are no branches arising from the aorta in TOF.     

Pulmonary damage following right ventricular outflow tachycardia ablation in a child: When electroanatomical mapping isn't good enough

A 14‐year‐old female was referred for severe pulmonary valve insufficiency after undergoing radiofrequency ablation for a right ventricular outflow tract tachycardia that originated in the proximal pulmonary artery at 10 years of age. Clinical records indicated that ablation was guided solely by electrograms and electroanatomical mapping. Due to myocardial tissue extensions, mapping failed to identify the level of the pulmonary valve annulus, which resulted in delivery of energy on the valve proper and into the pulmonary artery. She developed severe pulmonary valve insufficiency and moderate proximal pulmonary artery stenosis necessitating intravascular stent placement 4 years later with an associated transcatheter valve. Although the nonfluoroscopic approach during ablation has gained wide acceptance for use in children, this report highlights the benefits of adjunctive imaging to identify the precise location of the pulmonary valve when ablation therapy is contemplated in the right ventricle outflow tract.     

20例右室双出口临床分析

目的分析20例右室双出口矫治术的临床效果。方法 20例右室双出口患者室间隔缺损均为主动脉瓣下型,均采用心内隧道连接室间隔缺损与主动脉开口,自体心包片加宽右室流出道及肺动脉。结果全组无死亡,术后无左室流出道狭窄或右室流出道狭窄。随访3月-9年,临床效果满意。结论右室双出口解剖类型复杂,室间隔缺损的位置决定手术方案。手术的主要目的是重建左室流出道及右室流出道。     

法乐四联症根治术96例临床分析

目的 :总结 96例法乐四联症根治手术的临床经验。方法 :术中以间断褥式缝合绦纶补片修补室间隔缺损 (VSD) ,以经戊二醛处理的牛心包补片进行流出道成形增宽 ,其中 3 7例进行跨肺动脉瓣环成形。结果 :术后出现低心排血量综合征 9例 ,心律失常 7例 ;出血及心包填塞 4例 ;肺水肿 4例 ;肾功能不全 3例 ;胸腔积液 3例。术后死亡 3例 ,死亡原因、低心排 2例 ,室性心律失常 1例 ,死亡率 3 12 %。结论 :术中满意的室缺修补及流出道疏通成形 ,加上合理的围术期处理是法乐四联症根治术成功的关键。     

Intra-aortic balloon pump associated with dynamic left ventricular outflow tract obstruction after valve replacement for aortic stenosis.

An unstable patient with critical aortic stenosis had an intra-aortic balloon pump placed preoperatively for hemodynamic support and alleviation of symptoms. After separation from cardiopulmonary bypass following aortic valve replacement, the patient was hypotensive with increased pulmonary artery pressures. Transesophageal echocardiography revealed left ventricular outflow tract obstruction associated with systolic anterior motion of the mitral valve and severe mitral regurgitation. This pathophysiology was present when ventricular systole was preceded by balloon counterpulsation, but was absent during unassisted systole. This case report demonstrates a potentially significant untoward effect of intra-aortic balloon pump augmentation after aortic valve replacement for aortic stenosis. The timely diagnosis of this iatrogenic condition in the operating room permitted the prompt implementation of appropriate management strategies and avoided unnecessary surgical intervention.     

实时三维超声心动图对法洛四联症手术前后右室收缩功能分析

目的 应用实时三维超声心动图(RT-3DE)检测法洛四联症(TOF)手术前后右室收缩功能.方法 30例TOF患者常规二维超声心动图检查并确诊后,使用RT-3DE采集患者的右室全容积数据库,分析右室舒张末容量(RVEDV)、收缩末容量(RVESV),计算射血分数(EF).比较术前、术后12 d、术后3个月RVEDV、RVESV及EF值的变化.结果 30例患者中20%(6/30)患者术前右室EF低于50%,术后12 d收缩末容量较术前增加(P<0.05),EF值较术前减低(P<0.05),术后3个月RVEDV较术前增大(P<0.05).常规二维超声术后复查,70%患者(21/30)残留肺动脉分支狭窄,所有患者均存在肺动脉瓣反流.结论 RT-3DE能简便、准确地确定右室容积并计算右室收缩功能;TOF患者术后残留肺动脉分支狭窄及肺动脉瓣反流可能导致右室收缩功能下降.