一侧肺动脉起源于主动脉及伴发畸形的一期纠治

目的 探讨一侧肺动脉起源于主动脉及伴发畸形的纠治经验.方法 18例一侧肺动脉起源于主动脉及伴发畸形的患儿,其中15例为右肺动脉起源于主动脉,3例为左肺动脉起源于主动脉.2例为单纯一侧肺动脉起源于主动脉,另16例分别伴有主动脉弓中断、主动脉缩窄、动脉导管未闭、主肺动脉窗、法洛四联症、室间隔缺损、房间隔缺损、二尖瓣返流、三尖瓣返流、气管狭窄等心内外畸形.其中无手术指征和外伤感染各2例;1例放弃治疗;13例手术纠治.结果 13例手术患者无死亡病例,其中4例伴主动脉弓中断、主动脉缩窄患儿延迟关胸.术后3月～4年随访,除1例死于肺炎心衰外,余均存活,肺动脉压力下降,全组无主动脉瓣上狭窄及明显肺动脉分支狭窄.结论 一侧肺动脉起源于主动脉患儿易早期发生肺动脉高压,一经确诊需立即手术.手术方式首选与伴发畸形一期纠治.患儿尽管可有各种心内外伴发畸形,但早期手术纠治可获得较佳中长期疗效.     

主动脉弓中断伴复杂心内外畸形的一期纠治

目的介绍主动脉弓中断伴复杂心内外畸形的治疗。方法全组13例,平均(1.1±2.4)岁,平均体质量(7.3±7.2)kg。伴发畸形有完全性大动脉错位、右肺动脉起源于主动脉、第5主动脉弓残存伴狭窄、右心室双出口、永存动脉干、迷走右锁骨下动脉及左室流出道梗阻等。结果12例一期主动脉弓中断及伴发畸形解剖纠治;1例伴右心室双出口行主动脉弓吻合及肺动脉环缩。手术死亡3例,死亡率23.1%。随访中死亡1例,轻度吻合口梗阻3例。结论主动脉弓中断是一种少见的先天性心脏病,新生儿期死亡率高,围术期合适的干预是治疗成功的关键;婴幼儿早期易出现肺动脉高压,一经发现须及时手术。虽然手术成功率逐年提高,但由于伴发畸形复杂,远期生存率尚待长期随访观察。     

主动脉弓中断伴复杂心内外畸形的一期纠治

目的 介绍主动脉弓中断伴复杂心内外畸形的治疗.方法 全组13例,平均(1.1±2.4)岁,平均体质量 (7.3±7.2) kg.伴发畸形有完全性大动脉错位、右肺动脉起源于主动脉、第5主动脉弓残存伴狭窄、右心室双出口、永存动脉干、迷走右锁骨下动脉及左室流出道梗阻等.结果 12例一期主动脉弓中断及伴发畸形解剖纠治;1例伴右心室双出口行主动脉弓吻合及肺动脉环缩.手术死亡3例,死亡率23.1%.随访中死亡1例,轻度吻合口梗阻3例.结论 主动脉弓中断是一种少见的先天性心脏病,新生儿期死亡率高,围术期合适的干预是治疗成功的关键;婴幼儿早期易出现肺动脉高压,一经发现须及时手术.虽然手术成功率逐年提高,但由于伴发畸形复杂,远期生存率尚待长期随访观察.     

主动脉弓中断伴室间隔缺损的一期纠治

目的 探讨主动脉弓中断伴室间隔缺损纠治手术的经验.方法 36例主动脉弓中断伴室间隔缺损的患儿,其中1例伴轻度升主动脉发育不良和左室流出道狭窄,1例伴第5弓残存,2例伴主肺动脉窗,1例伴右肺动脉异常起源于主动脉.所有患者均行一期解剖纠治手术.结果 手术死亡2例,5例延迟关胸.术后6月～5年随访,1例死亡,2例左支气管轻度受压,2例存在心室水平残余分流,6例降主动脉流速稍快,全组心肺功能均良好.结论 主动脉弓中断伴室间隔缺损一经发现须及时手术治疗,一期纠治手术成功率较高,需密切关注主动脉吻合口狭窄及左支气管受压对患儿远期生存造成的影响.     

主动脉弓中断伴室间隔缺损的一期纠治

目的 探讨主动脉弓中断伴室间隔缺损纠治手术的经验.方法 36例主动脉弓中断伴室间隔缺损的患儿,其中1例伴轻度升主动脉发育不良和左室流出道狭窄,1例伴第5弓残存,2例伴主肺动脉窗,1例伴右肺动脉异常起源于主动脉.所有患者均行一期解剖纠治手术.结果 手术死亡2例,5例延迟关胸.术后6月～5年随访,1例死亡,2例左支气管轻度受压,2例存在心室水平残余分流,6例降主动脉流速稍快,全组心肺功能均良好.结论 主动脉弓中断伴室间隔缺损一经发现须及时手术治疗,一期纠治手术成功率较高,需密切关注主动脉吻合口狭窄及左支气管受压对患儿远期生存造成的影响.     

一侧肺动脉起源于主动脉的诊断及外科治疗

目的:总结一侧肺动脉起源于主动脉的诊断及外科治疗经验。方法:分析12例一侧肺动脉起源于主动脉患者的临床资料,其中右肺动脉异常起源主动脉9例,左肺动脉异常起源主动脉3例;病变为单纯一侧肺动脉起源异常6例,另6例伴有动脉导管未闭、室间隔缺损和法乐氏四联症。1例行动脉导管未闭结扎和异常起源肺动脉环缩术外,11例行矫治术,利用心包与人工材料补片(6例)、人工血管(4例)或同种管道(1例)连接。结果:早期死亡2例,死亡原因分别是术后低心排综合征和室性心律失常。术后随访8～84(48±19)月,晚期死亡1例,于术后3年死于右心功能衰竭。余均存活,无远期并发症,肺动脉瓣上、吻合口及肺动脉分支亦无明显狭窄。结论:超声心动图、右室造影和主动脉造影或超声心动图结合多排CT检查是诊断一侧肺动脉异常起源于主动脉的有效方法,手术方式首选与伴发畸形一期矫治手术矫治。     

主动脉移位术纠治完全型大动脉错位伴室间隔缺损和肺动脉狭窄的疗效观察

目的总结主动脉移位术纠治完全型大动脉错位伴室间隔缺损和肺动脉狭窄的临床疗效。方法 2005年1月—2010年6月,采用主动脉移位术纠治完全型大动脉错位伴室间隔缺损和肺动脉狭窄的患儿21例,其中合并流入道室间隔缺损4例,合并三尖瓣骑跨3例。对患儿手术情况和随访资料进行回顾性分析,评价疗效。结果院内死亡1例;其余20例患儿中,术后发生严重低心排血量综合征1例、急性肾功能衰竭7例、急性肝损伤1例、肺部感染2例,均经积极治疗后愈合出院。随访时间为8～61个月,无死亡病例;所有患儿无远期左心室流出道梗阻和室性心律失常发生,心功能均恢复正常。结论采用主动脉移位术纠治完全型大动脉错位伴室间隔缺损和肺动脉狭窄可取得良好的远期疗效,但术后早期对心功能影响较大,需要完善的监护治疗。     

主动脉弓中断的手术治疗

目的：回顾总结主动脉弓中断的手术治疗。方法：1998年10月－2000年9月，手术治疗主动脉弓中断6例。男性5例，女性1例，手术年龄2－9岁，体重10.5-31.0kg。所有病例术前均有反复呼吸道感染史，病例分型A型4例，B型1例，另1例为主动脉第5弓残存伴狭窄。2例经双切口方法手术，即通过左后外侧切口纠治主动脉弓中断，再经正中切口纠治心内畸形。4例均采用正中切口方法纠治主动脉弓中断和心内畸形。结果：全组死亡2例，均为术后发生严重肺动脉高压危象死亡。4例随访，最长12年，恢复良好。结论：主动脉弓中断采用正中切口方法，操作简便，暴露清楚，创面小，有利于术后恢复。由于该病早期出现肺动脉高压，一经诊断应及时手术纠治，手术在1岁内进行为好。     

正中切口一期纠治婴幼儿先天性主动脉缩窄伴室间隔缺损

目的　总结先天性心脏病主动脉缩窄伴室间隔缺损一期纠治的手术方法和临床经验。方法　1999年 1月～ 2 0 0 1年 12月 ,采用胸骨正中切口一期纠治 12例主动脉缩窄伴室间隔缺损患儿 ,手术年龄除 2例分别为 39和 2 9个月外 ,其余均小于 6个月 ;体重 2 .5～ 11.0kg ,平均为 (5 .2 4± 2 .77)kg。主动脉缩窄位于导管前型 6例 ,邻近动脉导管处 3例 ;主动脉弓发育不良伴主动脉缩窄 3例。 5例伴有动脉导管未闭 ;11例伴有室间隔缺损 ,其中 7例缺损位于膜周 ,4例位于肺动脉瓣下 ;1例伴有部分性房室隔缺损。手术采用胸骨正中切口 ,同时纠治主动脉缩窄和室间隔缺损。结果　死亡 1例 ,该例患儿年龄为 1个月 ,体重 2 .5kg ,外院转来时全身黄疸、慢性心功能不全 ,术后因心力衰竭而死亡 ,本组病死率为 8.3%。余 11例术后随访 5～ 30个月 ,恢复良好。结论　正中切口一期纠治婴幼儿先天性心脏病主动脉缩窄伴室间隔缺损 ,不仅可缩短手术时间 ,有利于术后心肺功能恢复 ,而且可同时纠治发育不良的主动脉弓     

常见先天性心血管畸形误诊分析

目的分析常见先天性心血管畸形误诊的原因,并总结临床经验。方法回顾分析2006年3月至2018年3月在中山大学附属第一医院心血管儿科就诊的误诊病例。所有病例均接受心电图、胸部X线、超声心动图检查。结果共收集7例误诊病例,其中心脏畸形1例,为部分性肺静脉异位引流伴房间隔缺损误诊为房间隔缺损;血管畸形6例,其中右肺动脉起源于升主动脉1例,左冠状动脉起源于肺动脉3例,动脉导管未闭合并心衰1例、先天性主动脉缩窄1例。所有误诊病例均有典型的影像学表现。结论临床医生严谨的态度、检查者规范的操作及两者间密切的沟通是有效避免误诊现象发生的关键。     

成人右肺动脉异常起源于升主动脉外科治疗2例报道

肺动脉异常起源于升主动脉(anomalous origin of pulmonary artery from ascending aorta,AOPA)是一种罕见的先天性心脏病,通常合并动脉导管未闭。AOPA患者多在出生后不久即出现症状,如不及时治疗,很少能活到成年。本文报道2例成人右肺动脉起源于升主动脉的病例,通过正确的诊断和适当的手术治疗,获得良好的早中期疗效。     

肺动脉起源异常的CT动脉造影诊断

目的评价计算机断层成像（CT）动脉造影诊断肺动脉起源异常的临床价值。方法对经CT动脉造影诊断肺动脉起源异常的9例患者进行回顾性分析,其中男6例,女3例;年龄0．2—33岁,年龄中位数2．8岁。复习相关的文献资料,进一步与超声心动图结果对照。CT动脉造影检查采用连续容积扫描。结果9例患者均经CT动脉造影诊断,其中8例为右肺动脉起源异常,1例为左肺动脉起源异常,均起自升主动脉。其中5例合并动脉导管未闭,3例合并室间隔缺损,1例合并主肺动脉间隔缺损,1例合并肺动脉瓣狭窄,1例为单发畸形。结论CT动脉造影在对肺动脉起源异常诊断中有较高的诊断价值,可以替代心血管造影等有创检查。     

Surgical treatment of anomalous origin of coronary artery from the pulmonary artery

Background Anomalous origin of coronary artery from the pulmonary artery is a rare congenital cardiac malformation with a mortality rate of up to 90% within the first year of life without surgical intervention. Direct implantation of the anomalous coronary artery （ACA） into the aorta is successful in early life, but it may have increased surgical difficulty and risk with age. This retrospective study summarized our operative experience in direct implantation for treatment of this coronary anomaly in pediatric and adult patients.
Methods From August 2000 to January 2003, 4 consecutive patients aged from 9 months to 41 years underwent dual coronary repair. Among them, two children and one infant with anomalous origin of the left coronary artery from the pulmonary artery （ALCAPA） and one adult was anomalous origin of right coronary artery from the pulmonary artery （ARCAPA）. Coronary arteries were directly implanted into the ascending aorta in 4 patients. In a boy with ALCAPA associated with moderate mitral insufficiency （MI）, whose ACA arose remotely from the ascending aorta, we created a tube-shaped graft using part of the pulmonary arterial wall in continuity with the origin of the left coronary artery （LCA）. Concomitant moderate MI was repaired in 2 patients, including this boy, after a dual-coronary repair.
Results All patients survived. There were no hospital or late deaths and no major complications as well. Echocardiography revealed that the left ventricular （LV） function including LV end-diastolic dimension （EDD） and ejection fraction （EF） was markedly improved at hospital discharge. At 3-6 years follow-up after surgery all patients were asymptomatic and currently in NYHA class 1.
Conclusions The best results are achieved with direct implantation of the ACA into the ascending aorta and simultaneous mitral valve repair if needed. Direct implantation is feasible in pediatric and adult patients with ALCAPA or ARCAPA including the coronary artery in a location remote from th     

冠状动脉异常起源于肺动脉的超声诊断及误诊分析

目的:探讨超声心动图在冠状动脉异常起源于肺动脉的诊断价值,并对误诊原因作分析。方法:对超声诊断为冠状动脉异常起源于肺动脉并手术证实的5例病例进行分析。结果:5例冠状动脉异常起源于肺动脉首次正确诊断全部由超声实现,其主要超声特征是:主动脉发出单支扩张冠脉;肺动脉发出异常分支并且血流倒灌;丰富冠脉侧支循环;常伴有左室长期心肌缺血继发表现。3例患者曾经进行超声心动图检查曾误诊为冠状动脉肺动脉瘘。结论:超声心动图诊断冠状动脉起源于肺动脉有非常重要的诊断价值。     

儿童左冠状动脉异常起源于肺动脉的手术治疗效果及随访

目的：总结左冠状动脉异常起源于肺动脉（ ALCAPA）的手术效果和随访结果。方法回顾性分析20例ALCAPA患儿的临床资料。术前左室射血分数（ EF）8％～65％,其中16例EF＜40％。20例患儿均在中低温体外循环下,移植左冠状动脉至升主动脉,其中8例行急诊手术,其余12例为亚急诊手术。6例同期行二尖瓣成形术。结果住院死亡3例（15％）,1例为术后低心排血量综合征,2例死于术后顽固性室颤。失访1例,另16例存活患儿无院外死亡,随访3～67个月。术前EF＜40％的患儿中有61．5％（8／13）在术后3～6个月期间EF升至50％以上;术前EF＜40％、随访1年以上的患儿中有90．9％（10／11） EF升至50％以上。结论外科手术移植左冠状动脉至升主动脉是治疗ALCAPA的有效方法。术后低心排血量综合征和恶性心律失常是其主要死亡原因。患儿如能安全度过围术期,心功能有望恢复正常,远期预后良好。     

Anomalous origin of left pulmonary artery branch from the aorta with Fallot＇s tetralogy： one case report

We report the case of a ten-year-old boy who had often presented with respiratory distress since born. Chest X-ray showed an enlarged right ventricle. Echocardiography demonstrated characteristic features of Fallot＇s tetralogy and the left pulmonary artery could not be visualized. However, cardiac catheterization disclosed that the left pulmonary artery had an anomalous origin in the ascending aorta. The patient underwent total surgical correction of the defects. Nine days later, he was discharged. We present the results of a literature review of the incidence, physiopathological, clinical, diagnostic, and surgical characteristics of this rare disease.     

Outcome of surgery for sinus of Valsalva aneurysm with discrete membranous subaortic stenosis

Background  Sinus of Valsalva aneurysm (SVA) is a rare cardiac anomaly, and SVA with discrete membranous subaortic stenosis is even rarer. The aim of the study was to make sure the incidence of SVA with discrete membraneous subaortic stenosis in SVA and their surgical results. We retrospectively analyzed 234 patients receiving surgical repair of SVA and reported the incidence of ventricular septal defect, aortic regurgitation, and discrete membranous subaortic stenosis. We also reported seven cases of SVA combined with discrete membranous subaortic stenosis and their surgical results.

Methods  Between January 1999 and December 2009, seven patients of SVA with discrete membranous subaortic stenosis underwent surgical repair of SVA and resection of subaortic discrete membrane. There were six male and one female patients. The mean age was (33.71±13.25) years (range 16–52 years). Associated cardiovascular lesions were aortic regurgitation (n=7), ventricular septal defect (n=5), coarctation of aorta (n=1), bicuspid aortic valve (n=1), patent ductus arteriosus (n=1), and aortic valve stenosis (n=1). The aortic valve was replaced in four patients and valvuloplasty was done in three. The other co-existing anomalies were corrected at the same time. All the seven patients were followed up from 18 to 125 months (mean (63.14±39.54) months). Among 234 SVA patients who underwent surgical repair, the number of cases with coexisting ventricular septal defect, aortic regurgitation, and discrete membranous subaortic stenosis was 129, 108, and 7, respectively.

Results  There was neither early death after operation nor late death during the follow-up period. All the seven patients were in the New York Heart Association (NYHA) functional classes I and II. There was no recurrence of discrete subaortic membrane during the follow-up period. The incidence of ventricular septal defect, aortic valve incompetence, and discrete membranous subaortic stenosis among 234 SVA patients was 55.13%, 46.15%, and 2.99%, respectively.

Conclusions  Surgical repair of SVA with discrete membranous subaortic stenosis showed good mid-term results. Resection of discrete subaortic membrane should be done actively while repairing SVAs. Long-term results need to be followed up.

     

冠状动脉起源异常29例

目的：了解冠状动脉起源异常（coronary artery origin anomalies,CAOA)的情况。方法：回顾性分析我院从1995至2001年2416例冠状动脉造影结果。结果：CAOA29例（1．2％），其中左前降支（LAD）和回旋支（LCX）分别独立起源于左冠状窦17例（58．6％），右冠（RCA）起源于左冠状窦6例（20．7％），RCA起源于升主支脉1例（3．4％），LCX起源于右冠状窦4例（13．8％）LCX起源于RCA1例（3．4％）；29例患者中除5例（17．2％）合并有冠状动脉狭窄外，其余24例（82．8％）患者未见冠状动脉狭窄。结论：CAOA是影响冠状动脉造影响的因素之一，但细胞观察左心室造影结果，选用不同型号的造影导管，终能满意地显示冠状动脉。最常见的类型是LAD和LCX分别独立起源于左冠状窦，大多数异常类型并不导致严重的临床症状，只有少数需要外科手术治疗。     

Anomalous origin of the right coronary artery: report of a case

A 63-year-old man was admitted with a complaint of dyspnea. Echocardiography showed severe aortic regurgitation (AR), and moderate mitral regurgitation (MR). Coronary angiography revealed that the right coronary artery (RCA) arose from the ascending aorta with a high takeoff and a significant stenosis at the distal segment of the RCA. Scintigraphy with Thallium showed a transient perfusion defect on the inferior wall. The diagnosis of AR and MR associated with anomalous origin of the RCA and myocardial ischemia was made. After successful catheter intervention for stenosis of the RCA, an operation was performed on the aortic and mitral valve. At surgery, the orifice of the RCA was located above the commissure of the right and left coronary cusps and the shape was obliquely elliptical. The RCA originated at an acute angle from the ascending aorta, and its proximal segment was incorporated in the wall of the aorta. After aortic valve replacement and mitral valve repair, a neo-ostium without unroofing of the intramural segment of the RCA was created at the proximal RCA, and the intima of the RCA was fixed to the intima of the aorta. The patient recovered uneventfully and is doing well without findings of myocardial ischemia at present 40 months after operation.