先天性心脏病的气囊血管成形术

本文就气囊血管成形术(BA)对主动脉缩窄和末稍性肺动脉分支狭窄的有效性,尤其是气囊径与狭窄度的关系及合并症等加以探讨。对象与方法1988年1月至1989年6月行BA的12例患者。其中主动脉缩窄(COA)6例(手术和非手术者各3例),未稍性肺动脉分支狭窄(PPS)5例,完全性大血管转位Mustard手术后出现的上腔静脉狭窄1例。方法:用穿刺法将气囊导管插入,把导线留在血管内,拨去套管后逆时钟方向转动,将导     

先天性心脏病介入治疗的适应证

先天性心脏病(先心病)的介入治疗最初作为一种姑息疗法以缓解完全性大动脉转位等重症发绀型先心病患儿低氧血症,即采用球囊房隔造口术替代外科开胸房间隔切开术,使患儿能够存活至进行根治术年龄。而后Porstmann应用泡沫塑料堵塞法进行动脉导管未闭(PDA)封堵术获得成功,使经导管治愈单一畸形先心病成为现实,也极大地促进先心病介入疗法的飞速发展。目前先心病介入治疗技术可分为两类:1.扩张术类:包括球囊房隔造口术、瓣膜成形术(球囊肺动脉瓣成形术、球囊主动脉瓣成形术)及血管成形术(主动脉缩窄血管成形术、肺动脉分支狭窄血管成形术)等。…     

先天性心脏病外科术后残余病变的介入治疗

目的 探讨先天性心脏病(先心病)外科术后残余病变介入治疗的可行性.方法 1995年2月 - 2010年12月收治98例先心病外科术后残余病变患儿,年龄9个月～ 16岁,平均(5.5 ± 3.8)岁.对有先心病手术残余漏患儿选择不同封堵器封堵治疗,对有血管狭窄的患儿做球囊扩张或支架植入治疗.结果 70例先心病手术残余漏患儿选择封堵器成功封堵.15例肺动脉分支狭窄者,12例选择NuMED CP覆膜支架植入,左、右肺动脉压力差(ΔP)分别降为4和5 mmHg,另3例因年龄小选择球囊扩张,但术后ΔP仅部分下降10 ～ 15 mmHg左右.6例主动脉残余局限性狭窄,其中5例球囊扩张,1例无效,4例ΔP下降至(19 ± 6.7)mmHg;另1例,用NuMED CP支架植入后ΔP仅3 mmHg.其他血管狭窄者选球囊扩张,ΔP均降至正常范围.结论 对于部分先心病外科术后残余病变,可以通过介入治疗完成,避免了再次开胸手术.     

经皮支架植入治疗心脏术后肺动脉分支狭窄1例

目的 运用经皮支架植入术治疗复杂先天性心脏病二期外科矫治术后残留的双侧肺动脉分支狭窄。方法 5岁女孩因患有肺动脉闭锁合并室间隔缺损分别在3岁和4岁时接受外科Blalock–Taussig分流术以及心内畸形矫治术。术后双侧肺动脉残留显著狭窄,通过经皮支架植入术在患儿右肺动脉和左肺动脉内植入直径分别为10mm与7mm的Genesis 支架。 结果 支架植入术后患儿临床以及血液动力学状况改善显著。右肺动脉和左肺动脉狭窄处内径分别由4.90mm和2.89mm增加至8.55mm和7.35mm,同时狭窄两端收缩期压力阶差分别由19mmHg和32mmHg下降至0mmHg。结论 通过经皮支架植入术,置入易于输送和扩张的Genesis 支架,可有效而安全地治疗小年龄儿童的肺动脉分支狭窄。     

Cheatham-Platinum支架置入术治疗儿童先天性心脏病血管狭窄的疗效评价

目的 评价NuMED Cheatham-Platinum(CP)支架置入术治疗儿童先天性心脏病(CHD)血管狭窄的即刻和早中期疗效.方法 2005年8月至2007年5月,采用CP支架置入治疗5例CHD血管狭窄(先天性主动脉缩窄1例,肺动脉狭窄4例).男3例,女2例,年龄4～15岁(中位年龄12岁),体重20～51 kg(中位体重24 kg).根据数字减影血管造影结果选择CP支架和NuMED双球囊,支架准确定位后先后充盈内外球囊扩张支架.结果 5例CHD血管狭窄进行了6次支架置入操作,共置入8只支架,均为8-zig CP支架,支架长度22～39 mm.除1例右肺动脉狭窄支架置入术中移位而于术后11个月重新置入第2只CP支架外,余4例6只支架即刻置入成功.跨狭窄段收缩压差由术前(43.43±25.61)mm Hg(1 mm Hg=0.133 kPa)降至术后(3.29±3.09)mm Hg(t=4.320,P<0.01),最窄处血管直径由术前(6.86±2.04)mm增加至术后(13.44±4.02)mm(t=-4.508,P<0.01).2例单侧肺动脉分支狭窄术后狭窄侧肺血流量占全肺血流量百分比分别由11.0%和13.0%增加至47.5%和52.2%,2例双侧肺动脉分支狭窄的右心室/主动脉收缩压比分别由术前62.3%和72.2%降至术后27.0%和33.3%.1例主动脉缩窄术后上肢血压由206/133 mm Hg降至156/95mm Hg.随访13～34个月(中位时间20个月),2只CP支架于术后6个月发生支架内再狭窄,余结果稳定无并发症发生.结论 CP支架置入术治疗儿童CHD血管狭窄安全可行,即刻和早中期疗效较好,但远期疗效有待进一步随访和更多病例的研究.     

Pul-Stent治疗先天性心脏病外科术后残留肺动脉分支狭窄的中长期随访

目的分析Pul-Stent治疗先天性心脏病患儿心脏外科术后残留肺动脉分支狭窄的安全性及有效性。方法回顾性研究。选择2014年8月至2015年6月因外科术后残留肺动脉分支狭窄在上海儿童医学中心行Pul-Stent植入的33例患儿为研究对象。分析患儿支架植入后的即刻疗效、随访结果及并发症情况。组间比较采用t检验。结果 33例患儿(男19例、女14例)均成功植入Pul-Stent(经皮导管31例、开胸直视2例), 共计植入35枚支架(小型19枚、中型15枚、大型1枚), 左肺动脉植入23枚, 右肺动脉植入12枚。植入时患儿年龄(6.8±3.1)岁, 体重(21.3±8.8)kg。初始扩张球囊直径范围6～16 mm, 29例(29/31, 94%)经皮植入患儿使用的输送长鞘为8～10 F。支架植入后即刻, 33例患儿支架植入处的肺动脉最窄直径从(4.0±1.7)mm增加到(9.1±2.1)mm(t=-21.60, P<0.001);26例经皮植入的双心室纠治术后患儿的跨肺动脉狭窄处的压力阶差由(30.5±12.3)mmHg(1 mmHg=0.133 kPa)降至(9.9±9.6)mmHg...     

主动脉缩窄的治疗进展

主动脉缩窄（coarctation of aorta，CoA）是一种常见的先天性疾病，指先天性主动脉局限性狭窄，病变部位管腔变小甚至闭塞，血流受阻，缩窄远近端形成侧支循环是其明显特征。过去 CoA 分为导管前型（婴儿型）和导管后型（成人型）。现在主张将 CoA分为单纯型（成人型）和复杂型，复杂型包括婴儿型（合并动脉导管未闭、室间隔缺损等畸形）和不典型型（合并主动脉弓发育不良或头臂动脉开口部狭窄以及部位不典型或多发狭窄）。未经治疗的 CoA 患者自然预后差，平均寿命约32～40岁。死亡原因包括充血性心衰（26％），主动脉破裂（21％），感染性心内膜炎（18％）和脑出血（12％）等致命性并发症［1］。CoA 的治疗目的是解除狭窄，重建主动脉正常血流通道，使血压和循环功能恢复正常，并尽可能减少并发症和缩窄周围的压力阶差。缩窄部位管腔直径减少超过50％和（或）压力阶差超过20 mmHg常被作为手术和介入治疗的指征［2］。治疗方法包括外科治疗、介入治疗以及镶嵌治疗。现就其治疗进展综述如下。     

周围肺动脉狭窄介入治疗的研究进展

周围肺动脉狭窄(PPAS)应用外科手术治疗可导致受治肺动脉进一步扭曲或狭窄,位于肺动脉分支远端难以暴露手术野而成为临床上较为棘手的问题.介入治疗是新近发展起来的一种治疗PPAS较理想的方法.PPAS介入治疗的发展先后出现了球囊扩张血管成形术、切割球囊血管成形术、血管内支架置入术三种方法,该文分别对三种方法的研究过程、治疗的禁忌证、适应证、并发症和有待改进的问题等进行概述,并对近年来在球囊治疗采用较保守的连续性扩张、输送系统的改进、支架应用的长度与再狭窄处理等方面的研究情况作一综述,以期促进对PPAS介入治疗的进一步研究.     

经皮介入治疗儿童主动脉缩窄疗效观察（附22例报告）

目的　评估儿童主动脉缩窄经皮介入治疗的安全性和有效性。方法　回顾性分析2007年2月至2017年5月在广东省人民医院心儿科确诊并接受了经皮介入治疗的22例18岁以下主动脉缩窄患儿的病例资料。对一般临床资料、超声心动图、心脏增强CT、心导管检查、相关并发症、随访结果和预后进行分析。结果　22例患儿中男13例, 女9例, 年龄9个月至17岁（8.7±5.7岁）, 体重9.5～80.0 kg［（30.7±20.0） kg］。16例主动脉缩窄行经皮球囊成形术,6例主动脉缩窄行经皮覆膜支架置入。缩窄处收缩压峰值压力阶差由术前（52.7±25.0） mmHg降至（9.7±11.4） mmHg（t＝8.326,P＝0.000,1 mmHg＝0.133 kPa）,缩窄处直径由术前（5.3±1.9） mm增加至（10.0±3.8） mm（t＝-6.996,P＝0.000）。2例术中出现局限主动脉夹层,随访3～6个月消失,无需干预;1例支架堵塞左侧锁骨下动脉需外科手术;无死亡病例。随访时间为3个月至9年,所有病例未行2次介入治疗或外科手术,支架未见变形及再狭窄。结论　对于治疗儿童主动脉缩窄介入的经皮球囊成形术和支架置入术的近中期疗效可靠,安全性高。球囊成形术对于外科术后再狭窄的患儿也是一种安全的、可接受的选择。     

肺动脉支架的应用现状及进展

目前,肺动脉狭窄的治疗方法有开胸手术、经皮球囊血管成形术、经皮支架植入术。30多年以来,支架植入治疗肺动脉狭窄发展迅速,很多情况下已经成为首选治疗方法。随着近年来可降解支架的出现,配合内外科镶嵌治疗概念的提出,经皮肺动脉支架植入更是显示出其广阔的临床应用前景。本文就经皮肺动脉支架的应用及发展进行综述。     

Dilatation von Klappen- und Gefäßstenosen

New catheter material and non-traditional access sites increased the efficacy of balloon dilation of stenosed valves and vessels. Right heart lesions such as critical pulmonary stenosis and severe pulmonary valve stenosis in childhood have a success rate of over 90%. In older children, the results of balloon dilation are excellent with a need for reintervention in less than 5% of cases and a very low mortality rate. Dysplastic pulmonary valves will need up to 45% surgical therapy. In case of Tetralogy of Fallot or complex lesions with severe pulmonary stenosis, balloon dilation of the right ventricular outflow tract can help to improve oxygen saturation. Dilatation of peripheral pulmonary arteries is associated with a high complication rate and the outcome is not always rewarding. Stent balloon implantation have a better efficacy and safety. Aortic valve stenosis with significant gradients in childhood are a clear indication for valvuloplasty. Excellent results are usually expected in 80 to 90% of patients. On the long term, valve replacement might still be warranted. In case of the critical aortic stenosis, the transvenous, antegrade access reduced the complication rate significantly. Comparison with surgical results are difficult, but the safety and mortality of both procedures seem to be comparable. Discrete native aortic coarctation as well as recoarctation in childhood can be dilated with a success rate of 65–90%. In the older patient stent implantations are preferred to balloon dilation. In the younger infants surgical management is advisable, because of very high recoarctation rate.     

An Infant with Subvalvar and Valvar Aortic Stenosis,Subvalvar and Valvar Pulmonary Stenosis,Severe Biventricular Hypertrophy and Pulmonary Hemorrhage: A Case Report

An infant with subvalvar and valvar pulmonary stenosis, subvalvar, and valvar aortic stenosis and hypertrophic cardiomyopathy, who presented with pulmonary hemorrhage, is reported. He had right ventricular hypertrophy, thickened pulmonary valve leaflets, severe asymmetric left ventricular hypertrophy with outflow tract obstruction, and a thickened and dysplastic aortic valve.     

Autologous Ross Operation for Congenital Aortic Stenosis

Congenital aortic stenosis is a relatively common cardiac anomaly encountered in approximately 5% of all children with heart disease. The Ross procedure is increasingly used for replacement of the aortic valve in children. We report a 12-year-old boy who was born with congenital aortic stenosis secondary to a bicommissural aortic valve. The patient underwent open valvotomy in infancy and aortic valvuloplasty 2 years later. Residual/recurrent stenosis prompted referral for aortic valve replacement, and he underwent an autologous Ross procedure, in which the aortic root was replaced with a pulmonary autograft and the repaired aortic valve was used to restore right ventricular-to-pulmonary artery continuity. The postoperative course was unremarkable. Nitroprusside, esmolol, and labetolol were used to control postoperative hypertension. He was discharged 4 days after surgery on oral furosemide and aspirin, and he has had no cardiovascular symptoms during follow-up. Recent echocardiography demonstrated mild right ventricular outflow tract obstruction with a peak velocity of 3.6 m/sec, with a gradient of 42 mmHg and moderate pulmonary insufficiency. There was no left ventricular outlet tract obstruction or aortic insufficiency.     

Tetralogy of Fallot with Aortic Stenosis: Repair Without Aortic Valve Intervention

A 14-day-old boy with tetralogy of Fallot (ToF), ductus-dependent pulmonary circulation, and significant aortic valve stenosis (AVS) underwent primary repair of ToF without aortic valve intervention. The pressure gradient through the aortic valve decreased spontaneously after the operation. This may have been due to the reduction of blood flow through the aortic valve postoperatively. For patients with ToF, AVS, and ductus-dependent circulation, ToF repair alone may alleviate the severity of the AVS.     

Current role of therapeutic heart catheterization in pediatric cardiology

OBJECTIVE: Cardiac catheterization has begun to be employed for therapeutic purposes since the middle sixties. In this paper the authors review the current indications, technique and results related to each of the interventional procedures. It also includes a brief sample of the Instituto Dante Pazzanese experience for illustrative purpose. METHODS: The main procedures that are addressed include balloon and blade atrial septostomy (Rashkind and Park procedures), pulmonary valvuloplasty, aortic valvuloplasty, mitral valvuloplasty (for rheumatic mitral stenosis), angioplasty for coarctation of aorta with or without stent implantation, angioplasty for pulmonary artery stenosis with or without stent implantation, percutaneous occlusion of the patent ductus arteriosus, percutaneous occlusion of atrial septal defects and balloon dilation of stenosed Blalock-Taussig shunts. The authors make a review of the most important papers published in the literature about Interventional Pediatric Cardiology, including their own experience. RESULTS: Good or excelent therapeutic results are achieved for pulmonic stenosis, coarctation of aorta, patent ductus arteriosus, atrial septal defects and pulmonary branch stenosis. Satisfactory palliations are obtained for aortic stenosis, rheumatic mitral stenosis and complex congenital heart diseases that require balloon atrial septostomy. CONCLUSIONS: Due to the recent technological development, pediatric therapeutic heart catheterization makes it possible to treat or palliate about 30% of all congenital or acquired heart diseases with encouraging results, sparing some patients from the need of cardiac surgery.     

Overview of interventional pediatric cardiology in 2004

Pediatric interventional catheterization is an expanding specialty with a range of mature, emerging, and investigative procedures and technologies. Many dysfunctional obstructions and/or shunts caused by congenital heart defects may be treated or significantly palliated in the catheterization laboratory. These include valvar pulmonary or aortic stenosis, the patent ductus arteriosus, coarctation of the aorta, branch pulmonary stenosis, atrial septal defects and even ventricular septal defects. Valve replacement technology, approaches to complex heart diseases such as single ventricle, and fetal interventions are subjects of active investigations. A comprehensive review of the present and future of interventional pediatric cardiology is presented.     

自体心包修复儿童主动脉瓣狭窄9例病例系列报告

目的：总结并分析以自体心包修复儿童先天性主动脉瓣狭窄的手术方法和早期预后。方法：纳入2013年7月至2015年6月在复旦大学附属儿科医院行自体心包主动脉瓣成形术的先天性主动脉瓣狭窄患儿,收集患儿的一般资料,围手术期情况,并发症,术前、术中、术后和随访时的超声心动图资料。结果：符合本文纳入标准的9例患儿进入本文分析,男6例,女3例,年龄4月龄至9岁。术前超声提示,三叶式和二叶式主动脉瓣分别为3例和6例;重度狭窄6例,中度狭窄1例,重度狭窄伴中度返流2例。9例均以自体心包再造或扩大主动脉瓣瓣叶。术后即刻与术前超声心动图比较：主动脉瓣最大跨瓣压差［（31.6±9.4）mm Hg vs（73.0±22.2）mm Hg, P=0.000］和主动脉瓣平均跨瓣压差［（15.8±18.3）mmHg vs （35.8±18.3）mmHg, P= 0.004］均下降。术后随访24～48（32±8）月,无死亡和再干预病例,未见严重不良事件,未见主动脉瓣重度狭窄或重度反流、升主动脉狭窄或扩张、主动脉瓣瓣环狭窄与扩张、瓣膜脱垂或瓣膜赘生物病例;3例患儿分别在随访18、24和12个月时出现瓣叶活动僵硬,瓣叶开放不完全。术后左室后壁厚径均呈下降趋势;末次随访时,8例患儿左室后壁厚径Z值（Z-LVPWd）均下降至正常水平（＜2）。结论：以自体心包修复主动脉瓣可改善先天性主动脉瓣狭窄患儿的血流动力学,手术风险低,术后早期疗效可,避免或延缓儿童主动脉瓣置换术,减少施行ROSS手术机会。     

常见先天性心脏病介入治疗进展及争论

先天性心脏病(CHD)的介入治疗近年来取得了很大进展,已成为CHD的一种重要治疗方法。然而目前人们对常见CHD介入治疗仍存在着一些争论。现重点讨论PDA、VSD、肺动脉瓣狭窄、主动脉瓣狭窄等常见CHD介入治疗适应证及方法学等方面的进展及争论。     

Transluminal dilatation of critical aortic stenosis and coarctation of a newborn infant aorta

We present a 20-day-old male infant with severe congestive heart failure. Echocardiography showed left ventricular hypertrophy secondary to critical aortic valve stenosis and severe coarctation of the aorta. The size of aortic annulus valve was 5.2 mm. At catheterization, the gradient of aortic valve stenosis was 104 mmHg and the gradient through aortic coarctation, measuring 14 mm in diameter, was 58 mmHg. Transverse and isthmic aorta showed moderate hypoplasia. After two dilatation procedures performed at an interval of 45 days, the pressure valve gradient decreased to 32 mmHg, that of the coarctation of the aorta decreased to 14 mmHg and the diameter increased to 5.4 mm.     

Left-sided anomalies in Ebstein's malformation of the tricuspid valve

Summary A review of 34 autopsied cases with classical Ebstein's malformation of the tricuspid valves revealed 8 cases with left-sided anomalies. Among these, appearing in 1 case each, were aortic atresia and persistent common atrioventricular canal. The latter 2 conditions were dominant clinically. In the remaining 6 cases the left-sided anomalies were not apparent clinically and probably of no functional significance. These conditions were parachute mitral valve, bicuspid aortic valve, cor triatriatum and pulmonary stenosis, cleft mitral valve, stenosis of individual pulmonary veins, and prolapse of mitral valve. This study was supported by Public Health Service Research Grant 5 RO1 HL05694 from the National Heart, Lung and Blood Institute.