体外循环下闭合重症未闭动脉导管体外循环的经验体会

<正> 动脉导管未闭(PDA)合并重症肺动脉高压,血管弹性较差者,用导管结扎或切断缝合术,可能发生导管破裂大出血。PDA 合并室间隔缺损或其它心内畸形者,经正中切口一期完成手术,用常规方法解剖游离动脉导管比较困难,出血危险性也较大,有些病例结扎动脉导管后,可能发生急性心衰。Kirklin 在1961年报告了在低温体外循环下,短暂停循环,经主肺动脉切口,直接缝合关闭 Potts 手术的吻合口,同期完成法乐氏四联症根治手术,取得成功,这一经验被以后的作者吸取应用。对于粗大的动脉导管,有肺动脉高压的,有钙化形成的,有感染性心内膜炎的,有动脉瘤形成的,高年龄的,再通的动脉导管,或合并其它心内畸形的动脉导管,用体外循环或深低温、停循环方法,经肺动脉或主动脉切口,直接缝闭动脉导管,取得较好结果,减少了常温手术下直接结扎缝合此类动脉导管出血的并发症。     

小儿双腔右心室的外科治疗

目的:探讨双腔右心室外科治疗方法。方法:外科手术治疗患者26例,平均年龄7.6岁,平均体质量25.4 kg,全组均伴有室间隔缺损,其中伴发房间隔缺损(ASD)15例,主动脉瓣下纤维嵴(SAS)6例,动脉导管未闭(PDA)3例,肺动脉狭窄(PS)1例。结果:所有患儿在体外循环下行手术治疗,手术顺利,全组无死亡,术后恢复良好。3年远期随访效果良好。结论:DCRV手术治疗关键在适当切除右心室内异常肌束和严密修补室间隔缺损,右心室流出道切口更有利于切除肥厚肌束,疏通右心室梗阻。     

室间隔缺损手术治疗70例临床分析

目的 :总结 1981年 12月至 2 0 0 2年 2月 70例先天性心脏病室间隔缺损手术治疗病例 ,探讨室间隔缺损的诊断、手术适应症、围术期治疗。方法 :采取中低温体外循环常规停跳修补室间隔缺损 5 4例 ,浅低温体外循环心脏不停跳修补室间隔缺损 16例 ;合并其它心内畸形 16例同期纠治。结果 :手术治愈 6 7例 ,重度肺动脉高压术后呼吸循环功能衰竭死亡 3例 ,治愈率 95 7%。结论 :室间隔缺损根据临床表现、心电图、X线胸片、超声心动图明确诊断 ,一经确诊应尽早手术 ,加强围术期治疗是保证手术成功的重要措施 ,临床手术治疗效果良好     

手术治疗动脉导管未闭四种术式的比较

198 6～ 2 0 0 1年 ,我院用四种手术方式治疗先天性心脏病动脉导管未闭 ( PDA) 112例。现报告如下 ,以探讨 PDA手术方式的合理选择。资料与方法 :112例 PDA患者中 ,男 46例 ,女 66例 ;年龄6个月～ 3 6岁。其中重度肺动脉高压 12例 ,中度肺动脉高压 17例。9例在体外循环低温下横行切开主肺动脉缝扎导管 ,其中 6例合并其他心脏畸形。64例为婴幼儿采用腋下纵行小切口切除部分第 4肋骨 ,纵行切开纵隔胸膜结扎导管。16例采用后外侧小切口胸膜外结扎动脉导管。2 3例年龄较大患者采有左胸后外侧切口结扎导管。结果 :112例患者无手术中和手术后…     

小切口不停跳心内直视手术的临床研究

目的 :探讨小切口不停跳心内直视手术治疗的方法和意义。方法 :对 1997年 10月至 2 0 0 3年 6月进行的 4 82例小切口不停跳心内直视手术进行回顾性分析。其中继发孔房间隔缺损 (ASD) 193例 ,合并三尖瓣关闭不全 (TVI) 4 0例 ,合并动脉导管未闭 (PDA) 16例 ,合并室间隔缺损 (VSD) 4 5例 ,合并部分肺静脉异位引流 (PAPVD) 7例 ;VSD 2 12例 ,合并右室双腔心 (DCRV) 8例 ,PDA 11例 ;肺动脉瓣狭窄(PS) 4例 ;法洛三联症 6例 ;法洛四联症 (TOF) 3例 ;二尖瓣狭窄伴关闭不全 (MVD) 4 1例 ;左房黏液瘤(LAM) 8例。结果 :全组早期死亡 3例 ,死亡率 0 6 % (3 4 82 ) ,术后抗凝过量出血、低心排综合征、严重肺动脉高压各 1例 ,均与手术方式无关。结论 :微创小切口心内直视手术创伤小、美观、恢复快、安全有效 ,但应严格掌握手术适应证及合理选择手术切口     

42例动脉导管未闭外科治疗

目的 回顾分析42例动脉导管未闭外科（patent duct arterious,PDA）治疗效果。方法 ①单纯PDA或同时合并心内畸形,动脉导管小～中,可采用左前或左后外侧胸切口或胸部正中切口结扎（30例单纯PDA,3例合并心内畸形）;②动脉导管粗大、钙化或同时合并其他心内畸形,胸部正中切口体外循环（cardiopulmonary　bypass,CPB）下经肺动脉切口闭合（9例）;③同期或分期矫治心内畸形。结果术中死亡1例（死于低心输出量综合征）。其他手术并发症包括小量残余分流2例、短暂声音嘶哑2例、乳糜胸2例。除2例残余分流病例外,其余病例无导管再通,心功能明显改善,痊愈出院。结论单纯结扎简便易行,适用于大多数病例：导管粗大、重度肺动脉高压、怀疑有动脉内膜炎、钙化、合并其他心内畸形病例,应采用CPB（结合深低温、低流量）下缝闭,以策安全。     

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。     

经导管介入治疗婴幼儿大型动脉导管未闭临床疗效

目的 探讨应用导管介入治疗直径≥5 mm婴幼儿大型动脉导管未闭(patent ductus arteriosus,PDA)的临床疗效.方法 回顾性分析60例大型PDA(直径≥5 mm)患儿的临床资料,着重分析手术方法、临床疗效和随访结果.结果 56例PDA采用Amplatzer或国产先健动脉导管封堵器,3例采用膜部室间隔缺损封堵器,1例采用肌部室间隔缺损封堵器.封堵成功率为98.3%(59/60),术后1 d超声心动图显示15%(9/60)微量至少量残余分流,术后3月超声心动图复查未见残余分流;3例采用室间隔缺损封堵器患儿术后12月超声心动图示左肺动脉血流速度增快.结论 应用导管介入治疗直径≥5 mm婴幼儿大型PDA是安全、有效的方法.     

室间隔缺损合并动脉导管未闭并发肺动脉高压23例报告

1996年3月至2003年3月，我们共手术治疗室间隔缺损(VSD)合并动脉导管未闭(PDA)并发肺动脉高压(PH)23例。现报告如下。     

同期手术治疗动脉导管未闭合并心内畸形8例分析

目的总结动脉导管未闭合并其他心脏畸形的外科治疗经验,以提高手术效果。方法8例PDA合并心内畸形的病人,5例行经胸骨正中切口体外循环插管前动脉导管结扎术,3例行经胸骨正中切口体外循环转流后经肺动脉切口动脉导管缝闭术。结果本组无早期死亡病例,术后均恢复顺利,治愈出院,随访4个月至5年,无晚期死亡和导管再通。结论同期手术矫治动脉导管未闭合并心内畸形,可以减少手术风险和两次手术创伤的痛苦。     

Complete repair of the interrupted aortic arch by an anterior approach. Apropos of 9 cases

Interrupted aortic arch is a duct-dependent heart disease, and this is why the use of prostaglandins E, which keep the ductus arteriosus open to feed the descending aorta, has transformed its formerly sombre prognosis. The authors report the cases of 9 neonates and infants presenting with interrupted aortic arch associated with ventricular septal defect who underwent total repair of the malformations under extracorporeal circulation. Five children were less than 10 years' old. The aortic lesions were of type A in 2 cases (interruption distal to the left subclavian artery) and of type B in 7 cases (interruption between the left carotid and subclavian arteries). In the second group the lesions were of type B1 in 5 cases (without retro-oesophageal right subclavian artery) and of type B2 in 2 cases (right retro-oesophageal artery distal to the interruption). There was one death (11 p. 100). So far, no child has been reoperated upon for residual aortic obstruction or reopened ventricular septal defect. This technique of total repair in one stage, with closure of the ventricular septal defect, seems to be preferable to the two-stage technique in which stage one includes, in addition to aortic arch repair, cerclage of the pulmonary artery, a procedure with numerous disadvantages and a higher overall mortality rate.     

Patent ductus arteriosus closure using an Amplatzer? ventricular septal defect closure device

The ductus arteriosus originates from the persistence of the distal portion of the left sixth aortic arch. It connects the descending aorta (immediately distal to the left subclavian artery) to the roof of the main pulmonary artery, near the origin of the left pulmonary artery. Persistence of the duct beyond 48 h after birth is abnormal and results in patent ductus arteriosus (PDA). PDA is rare in adults because it is usually discovered and treated in childhood. Mechanical closure remains the definitive therapy because the patency of ductus arteriosus may lead to multiple complications, depending on the size and flow through the ductus. PDA closure is indicated in patients with symptoms and evidence of left heart enlargement, and in patients with elevated pulmonary pressures when reversal is possible. Transcatheter closure is the preferred technique in adults because it avoids sternotomy, reduces the length of hospital stay and is associated with fewer complications compared with surgery. First demonstrated in 1967, both the technique and the occluder devices used have since evolved. However, designing an ideal PDA occluder has been a challenge due to the variability in size, shape and orientation of PDAs. The present article describes a case involving a 35-year-old woman who presented to the Center for Advanced Heart Failure (Houston, USA) with congestive heart failure due to a large PDA, which was successfully occluded using an Amplatzer (St Jude Medical, USA) muscular ventricular septal defect closure device. The wider waist and dual-retention discs of these ventricular septal defect closure devices may be important factors to consider in the future development of devices for the occlusion of large PDAs.     

Truncus arteriosus malformation: a spectrum including fourth and sixth aortic arch interruptions

Previous explanations of the pathogenesis of truncus arteriosus malformation have emphasized absence of conotruncal ridges, absence of pulmonary conus, or fusion of ventricular outflow streams. These concepts explain the persistence of the single semilunar valve and outflow vessel, but have not elucidated the significance of many associated anatomic lesions. We studied the 19 patients with truncus arteriosus malformation listed in the autopsy files of The Johns Hopkins Hospital whose hearts were available for review. The patients ranged in age from one day to 30 years, and included 12 males. All hearts showed a single semilunar valve with three or four cusps, and a high ventricular septal defect. In 13 patients, the aorta was larger in diameter than the pulmonary artery, and no remnant of the ductus arteriosus was present (interruption of the embryonic sixth arch). In one patient, the aorta was larger than the pulmonary artery, and a small, patent ductus arteriosus was present. In two patients, the pulmonary artery was larger than the aorta, and the aortic arch was interrupted. The remaining three cases could not be fully evaluated for the status of the ductus arteriosus and size of the great arteries. We tabulated the number of associated anatomic lesions which were unexplained by each of the three pathogenetic hypotheses. The fused outflow stream hypothesis, which explains truncus arteriosus malformation as the result of a maldirection of ventricular outflow streams so that the separation of two semilunar valves is prevented, was superior at the 0.05 level of significance. This concept explains the spectrum of great vessel patterns in which flow is dominant into either the sixth or fourth embryonic aortic arch, permitting early disappearance of unperfused segments of the aortic arches, and is commonly seen at autopsy as either an interrupted aorta or an absent ductus arteriosus.     

Interrupted aortic arch. A series of 15 patients

Between 1983 and 1989, 15 children underwent surgical repair of interrupted aortic arch at 1 to 20 days of age. The anatomical form was a Celoria and Patton type B in all patients with an associated perimembranous ventricular septal defect in all but one who had multiple ventricular septal defects, and patent ductus arteriosus. Six children had a retro-esophageal right subclavian artery, two had subaortic stenosis and two had a right-sided descending thoracic aorta. In two children with severe hypoplasia of the ascending aorta the repair was performed in one stage with two deaths due to left ventricular failure. In the other B cases, a two-stage repair was carried out. The reconstruction of the aortic arch varied according to the individual case. All children had pulmonary artery banding. Seven children survived longer than 30 days. Six of them later underwent a complete repair. The only survivors were those patients in whom the neo-aortic arch grew harmoniously. The authors conclude that: a two-stage repair gave disappointing results in this series of consecutive patients, mainly because of the poor quality of the reconstruction of the aortic arch by thoracotomy.     

主肺动脉窗及合并心脏畸形的外科手术治疗经验总结

目的:回顾分析主肺动脉窗及合并心脏畸形的临床特征,总结外科手术治疗的经验和效果.方法:自1997-01至2011-05,共有46例主肺动脉窗患者接受了外科手术治疗,平均年龄(3.2±2.5)岁(0.1～16)岁,平均体重(11.4±6.2)kg(4～47)kg.合并的心脏畸形包括右肺动脉起自升主动脉7例、二尖瓣关闭不全7例、主动脉瓣下隔膜5例、动脉导管未闭6例、主动脉弓中断5例、室间隔缺损4例、房间隔缺损4例、右室双出口3例、三尖瓣关闭不全2例、右冠状动脉起自肺动脉2例、法乐四联症1例.43例患者在体外循环下手术,其中23例经主动脉切口补片修补,12例经肺动脉切口修补,4例经主肺动脉窗修补,2例行切断缝合.其余3例患者在非体外循环下行主肺动脉窗缝扎(1例)或切断缝合(2例).合并心脏畸形同时矫治.结果:早期死亡3例,平均随访5年,1例患者术后一年死于肺动脉高压,其余患者无再次手术及残余分流,主动脉和肺动脉发育正常.结论:主肺动脉窗一经诊断,应尽早外科手术治疗,体外循环下经主动脉切口补片修补是首选的方法.     

Echocardiographic assessment of interrupted aortic arch

BACKGROUND: In patients with interrupted aortic arch echocardiography provides detailed information about the anatomy of the aortic arch and the associated cardiac anomalies. Only a few reports have evaluated the reliability of this non-invasive diagnostic procedure by correlation with angiographic and surgical findings. METHODS: From 1988 through 1993, 45 infants with interrupted arch underwent surgical repair (mean age 13.02 days). Of the patients, 33 had interruption of the arch between the left common carotid and subclavian arteries; 25 patients had a ventricular septal defect, and the remaining 20 had coexisting complex congenital heart defects. Preoperative diagnosis was made exclusively by echocardiography in 25 of the patients. Accuracy of echocardiographic diagnosis was evaluated retrospectively by comparing preoperative studies with angiography and surgical reports. We then investigated whether the morphologic features of the interrupted arch might influence surgical procedure or outcome. RESULTS: Intracardiac anatomy was accurately diagnosed by echocardiography in all cases; in 2 patients angiography provided additional information concerning the morphology of the aortic arch. Operative notes described differences in morphology of the arch in 7 patients, but these did not influence the surgical procedure. Direct anastomosis of the interrupted segments was possible in 38 patients, and 36 patients underwent primary intracardiac repair. Echocardiographic measurements revealed that the diameter of the ascending aorta was related to the number of vessels originating from the proximal aortic arch. The distance between the interrupted segments was significantly different according to the site of interruption, but not between cases with an isolated ventricular septal defect versus those with complex heart disease. It did not influence the method of arch repair, nor was it related to recurrent or residual obstruction. CONCLUSION: Preoperative echocardiography offers accurate and complete diagnosis in the critically ill neonate with interrupted aortic arch and associated intracardiac abnormalities.     

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.     

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)     

One-stage repair of cardiac and arch anomalies without circulatory arrest

Between 1999 and 2002, 23 patients underwent single-stage complete repair of cardiac anomalies and aortic arch obstruction, without circulatory arrest. Median age was 1.2 years. Intracardiac defects included ventricular septal defect in 9, double-outlet right ventricle in 6, d-transposition of the great arteries and ventricular septal defect in 2, subaortic obstruction in 3, and atrial septal defect in 3. Fourteen patients had coarctation of the aorta, 6 had coarctation with hypoplastic aortic arch, and 3 had interrupted aortic arch. Simple techniques were employed such as cannulation of the ascending aorta near the innominate artery and maintaining cerebral and myocardial perfusion. After correction of arch obstruction, intracardiac repair was undertaken. The mean cardiopulmonary bypass time was 169 min, aortic crossclamp time was 51 min, and arch repair took 16 min. There was no operative mortality or neurological deficit. In follow-up of 1-43 months, no patient had residual coarctation. This simplified technique avoids additional procedures, reduces ischemic time, and prevents problems related to circulatory arrest.     

Accuracy of two-dimensional echocardiography in the diagnosis of congenital heart disease

To assess the accuracy of 2-dimensional (2-D) echocardiography in the evaluation of cardiac anatomy in patients with congenital heart disease, 2-D echocardiograms were performed in 126 infants and children before cardiac catheterization and angiocardiography. The segmental echocardiographic analysis included determination of intracardiac, great artery, systemic venous and pulmonary venous anatomy. The 126 patients had 259 separate cardiovascular abnormalities, of which 226 (87%) were prospectively identified by 2-D echocardiography. There were 8 false-positive diagnoses. The most common lesions and the sensitivity and specificity of echocardiography were: patent ductus arteriosus, 41 patients (83% and 100%, respectively), ventricular septal defect, 35 patients (86% and 100%); atrial septal defect, 26 patients (85% and 99%); pulmonary valve stenosis, 25 patients (77% and 97%), transposition of the great arteries, 16 patients (100% and 100%); and total anomalous pulmonary venous connection, 14 patients (85% and 100%). Less common defects and their rate of detection included coarctation of the aorta, 10 of 12 patients; atrioventricular canal, 10 of 10 patients; tetralogy of Fallot, 10 of 10 patients; aortic valve stenosis 8 of 8 patients; right aortic arch, 8 of 8 patients; interrupted aortic arch, 4 of 4 patients; and unilateral pulmonary vein atresia, 0 of 1 patient. In 33 patients (26%), the errors in echocardiographic analysis were judged to have surgical importance. Most errors were the result of overlooking or misinterpreting data that had been appropriately recorded on videotape. Pulmonary valve stenosis and patent ductus arteriosus are the lesions most likely to be misdiagnosed by ultrasound studies relying on imaging alone.