CASE REPORT OF A 6-WEEK-OLD BOY WITH BLAND- WHITE-GARLAND SYNDROME

Bland-White-Garland syndrome is a rare syndrome with anomalous origin of the left coronary artery （ LCA ） arising from the pulmonary artery, resulting in left ventricular failure. It could occur shortly after birth. We here reported the case of a 6-week-old boy with aortostenosis. Coronary angiography revealed an anomalous LCA arising from the pulmonary artery. Representation of a prominent right coronary artery （ RCA ） delivered numerous collateral vessels to the LCA area, The patient underwent a correction operation with translocation of the LCA and re-implantation into the ascending aorta. One month after operation, clear decrease in the expanded ventricle was noted with an increase in the contractibility.     

Percutaneous closure of left intemal mammary artery-to-pulmonary artery fistula using the domestic vascular plug： a case report

A 55-year-old man was admitted for transcatheter closure because of pulmonary arteriovenous fistula by magnetic resonance angiography （MRA）. He had a history of occasional chest pain more than one year. Angiography didn＇t reveal significant stenosis at coronary artery. The patient was found a continuous grade 2/6 murmur over the left upper parastenal area one month ago. Chest MRA revealed a possible left superior pulmonary arteriovenous fistula. For diagnosis and localization of the fistula, aortography and selective angiography of the intemal mammary artery was performed and presented a left internal mammary artery-to-pulmonary artery fistula. The fistula was successfully closed using an 12 mm domestic vascular plug. Chest MRA showed that the fistula disappeared at two-month follow-up     

Bridging bronchus complicated with a left pulmonary artery sling a rare entity

To the editor： The bridging bronchus （BB） is a rarely reported congenital anomaly in which right middle and lower lobes are supplied by an accessory bronchus arising from the left main bronchus,1 A sling left pulmonary artery （SLPA） is also a rare congenital cardiac malformation, in which the left pulmonary artery arises from the right pulmonary artery and passes between the trachea and esophagus, causing tracheal stenosis by the pulsating pulmonary artery.： Here we present a rare pediatric case of BB complicated with a SLPA.     

Anomalous origin of left coronary artery from pulmonary artery. Successful correction for a 7-year-old patient.

A 7-year-old girl with an anomalous origin of the left coronary artery from the pulmonary artery restored connection of the left coronary artery to the ascending aorta by aortocoronary bypass graft. Aortocoronary bypass grafting was performed with a vascular prosthesis. Postoperative angiography showed a patent left coronary artery without narrowing or kinking. Clinical improvement was rapid and the left ventricular function recovered completely.     

Multi-detector computed tomography evaluation of tracheobronchial anomaly in pediatric patients with left pulmonary artery sling

The left pulmonary artery sling (LPAS) is a rare vascular anomaly causing respiratory distress in which the left pulmonary artery arises from the posterior aspect of the right pulmonary artery,courses posteriorly to the right of the bronchus and passes between the trachea and oesophagus to reach the hilum of the left lung.The LPAS is frequently associated with tracheobronchial tree anomalies and congenital cardiac defects.Proper assessment of the tracheobronchial and cardiovascular anomaly is essential in LPAS for planning management of the patient.Currently,the most important prognostic factor is considered to be an associated tracheobronchial tree anomaly.1 The purpose of this study was to demonstrate the role of multi-detector computed tomography (MDCT) for diagnosing LPAS and assessing the anatomical relationship with tracheobronchial tree anomaly.     

Transcatheter closure of a giant coronary artery fistula with patent duct occluder

Coronary artery fistula （CAF）, an uncommon form of congenital heart disease, is characterized by abnormal communication between a coronary artery and a cardiac chamber or vessel. This disease is often found incidentally in asymptomatic individuals, and leads to cardiac failure, myocardial ischemia and angina, infective endocarditis, and heart rupture in later life. Both surgical repair and transcatheter closure were effective and safe in the treatment of CAF, but percutaneous management of CAF can obviate median sternotomy and cardiopulmonary bypass, and there may be less morbidity with transcatheter embolization techniques, including the use of occluders or microcoils. We report a successful percutaneous closure of a giant CAF from the left coronary artery to the right ventricle using patent duct occluder.     

An in vivo model of in situ implantation using pulmonary valved conduit in large animals under off-pump condition

Background The application of pulmonary valved conduit to reconstruct the continuity between right ventricles and pulmonary artery is one of the major surgeries.This study aimed to establish an in vivo model of in situ implantation using pulmonary valved conduit in large animals under off-pump condition to validate the long-term effects of artificial pulmonary valved conduit.Methods Domesticate juvenile male sheep and tissue-engineered poorine pulmonary valved conduit were used for the experiment：30 sheep,weighing （15±3） kg （range 13 to 17 kg） were randomly divided into two groups which were all operated under general anesthesia by off-pump surgery （group 1） and left thoracotomy （group 2）.Two different off-pump surgical methods were used to perform cannulation in sheep pulmonary artery to replace part of sheep pulmonary artery with pulmonary valved conduit which will work together with sheep pulmonary artery and valves.During the experiments,animal survival,complication rates,operating time and blood loss were recorded to compare the results between groups and to establish a surgical method with minimal invasion,simplicity,safety,and high success rates.Results In group 1,a total of 15 cases of surgeries were performed,in which two sheep died; the operative mortality was 13.3％ （2/15）.In group 2,a total of 15 cases of surgeries were performed,and the surgical mortality rate was 0 （0/15）.The operation time and blood loss in group 2 was significantly better than that in group 1.The postoperative echocardiograms showed that,after the surgeries by these two methods,the blood flows were normal,and the valves can open and close freely.Autopsy after 6 months showed that the inner wall and the valves of pulmonary valved conduit were smooth with no thrombus formation.Conclusion These two off-pump methods are feasible and safe with fewer traumas; but the second method is better and particularly suitable for the establishment of a juvenile animal model.     

Congenital absence of right pulmonary artery associated with left patent ductus arteriosus and aortic coarctation

To the editor:A 7-month-old appeared with acyanosis,an Ⅲ/6 continuous murmur at left sternal border and weak femoral pulses.Electrocardiography presented biventricular enlargement with strain.Chest X-ray showed asymmetric bilateral pulmonary blood flow.Echocardiography revealed absence of right pulmonary artery (RPA),aortic coarctation (CoA),patent ductus arteriosus (PDA),moderate to severe tricuspid regurgitation,enlargement of right atrium and right ventricle.Cardiac catheterization and angiogram were performed under general anesthesia.The main pulmonary artery was extended only by the left pulmonary artery (LPA).A small isolated RPA was shown by right pulmonary vein wedge angiography,and there were fine collateral vessels into the right lung firom right brachiocephalic artery and the descending aorta.The right ductus arteriosus closed with a diverticulum left in the base of the innominate artery in this left aortic arch.The long tube-shaped left ductus was patent with 3 mm diameter,and there was preductal discrete aortic coarctation with systemic pressure gradient 34 mmHg (Figure 1):QP/QS was 1.25 and PVR was 8.2 wood unit.     

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     

Pulmonary hypertension with a huge thrombosis in main stem of pulmonary artery

A huge thrombosis in the main stem of the pulmonary artery (PA) with pulmonary hypertension has rarely been reported. We present two cases diagnosed and treated in our hospital. One suffered from polyarteritis with a huge thrombus in PA revealed at autopsy. The second case had congenital heart disease of the patent artery duct; and the huge thrombus was found on echocardiogram and extirpated in surgery.     

肺癌侵犯大血管的外科处理

目的探讨大血管成形术在肺癌外科中的应用价值.方法回顾性分析136例肺癌手术中涉及大血管成形术患者的病例资料.TNM分期Ⅱb 29例;Ⅲa 45例;Ⅲb 62例.鳞状细胞癌98例,腺癌31例,小细胞肺癌7例.结果 136例中上腔静脉置换或部分切除术18例,血管旁路分流术4例;肺动脉成形术92例,其中右上叶肺动脉袖状切除2例,部分切除12例,左上叶袖状切除14例,部分切除64例;左心房部分切除22例.无手术死亡者,随访结果满意.结论应用大血管成形术可以提高手术切除率及手术的安全性,可以最大限度地保留患者的肺功能,并有助于提高患者的术后生存率及生活质量.     

Anomalous origin of the left coronary artery: the effects of aortocoronary vein bypass on left ventricular function

The diagnosis, angiographic evaluation and surgical treatment by aortocoronary vein bypass are described in a 3½-year-old girl with anomalous origin of the left coronary artery from the pulmonary artery. The anomaly had resulted in cardiac dilatation, diminished left ventricular contractility, an aneurysm of the left ventricular free wall and mitral regurgitation.

At the postoperative cardiac catheterization the graft was demonstrated to be patent, but a significant proportion of the flow to the left coronary artery was derived from anastomotic connections with the right coronary artery. The most striking evidence of improvement was obtained from the left ventricular volume studies which showed that the end systoiic volume had decreased from 85 to 49 ml./m.² with an increase in ejection fraction from 0.39 to 0.62, suggesting enhanced left ventricular contractility after surgery.

The patient continues to do well and is free from symptoms.

     

肺血管成形术治疗支气管肺癌

本文对9例支气管癌病人进行了肺血管成形术。1例左肺上叶支气管袖状切除术并左肺动脉袖状切除成形术,2例右肺上叶支气管袖状切除并肺动脉楔形切除吻合,1例病人癌肿侵及肺上静脉,切除部分受侵的静脉壁,用奇静脉片成形术,5例行肺动脉部分切除吻合术。对手术的方法,血运阻断时间及再建顺序进行了讨论。     

Mycotic aneurysm of the left subclavian artery: CT findings

Mycotic aneurysms caused by aspergillosis are rare. We report a nine-year-old girl with acute lymphoblastic leukaemia who had invasive pulmonary aspergillosis and subsequently developed a left subclavian artery aneurysm. Prior to the aneurysm, computed tomography (CT) of the chest showed a nodule with an air crescent in the left upper lobe, adhering to the mediastinum and the left subclavian artery. The left subclavian artery was ill-defined and had a small lumen, and it was embedded in the wall of the nodule. 37 days after the chest CT, the patient underwent a left thoracotomy because of massive haemoptysis, at which time a false aneurysm in the left subclavian artery was found. Plication of the aneurysm was performed. On a follow-up CT with multiplanar reconstruction six days after surgery, there were the plicated aneurysm and a small amount of pleural effusion in the upper portion of the left hemithorax, adjacent to the plication. In invasive pulmonary aspergillosis, it is important to be aware of the possibility of mycotic aneurysms, particularly in patients with pulmonary lesions adjacent to mediastinal vessels with ill-defined borders and small lumens, since the aneurysms may increase in size and rupture. CT, particularly multidetector CT, helps in visualisation of mycotic aneurysms.     

肺动脉闭锁合并室间隔缺损不同术式的疗效分析

目的:比较分析不同术式在肺动脉闭锁合并室间隔缺损（PA/VSD）治疗中的手术效果。方法:回顾性分析2006年9月至2017年9月天津市胸科医院心外科诊治的25例B型肺动脉闭锁合并室间隔缺损患儿（者）的临床资料。其中男16例,女9例;年龄2d～26.4岁;体质量3.5～46.0 kg。根据选取的手术方式的不同,将25例病人分为两组:姑息手术组和根治手术组。收集两组患儿（者）术前、术中和术后的各项相关资料和指标进行比较分析。结果:术后早期死亡2例（6.9%）,均来自根治组。两组死亡差异无统计学意义,P=0.07。死亡原因1例考虑为呼吸衰竭,另1例为低心排综合征。术后肺部感染3例,吻合口出血行二次开胸止血1例,手术切口愈合不良1例,术后左肺动脉较术前狭窄1例。术后随访10个月～8年,失访1例。随访期间,测定肺动脉指数均有不同程度的增加。姑息组中共有5例最终完成根治术。结论:不同术式治疗肺动脉闭锁合并室间隔缺损安全有效。早期建立肺动脉前向血流对于肺动脉的发育有重要的促进作用。术前应综合评估肺动脉的形态发育和侧支血管的解剖情况,选择相应的个体化的手术方式。     

主动脉缩窄的外科治疗

作者报告二例主动脉缩窄的手术治疗,一例采用左锁骨下动脉片主动脉成形术,合并大血管畸形一例采用左锁骨下动脉远端与降主动脉吻合术。术后均获满意疗效,随访2～20个月均无重大并发症。作者对主动脉缩窄手术方法选择,合并心衰、肺动脉高压的主动脉缩窄手术术后处理进行了讨论。     

An unusual case of dual coronary artery fistulas to main pulmonary artery

Coronary artery fistula is an anomaly in which a coronary artery directly connects to a cardiac chamber or great vessel. Its incidence is around 0.1 to 1% in the adult population. Dual coronary artery fistulas are far less common and their incidence is estimated to be around 5% in patients with this anomaly. Closure of the fistulas is indicated in patients with myocardial ischemia, large left to right shunt, congestive heart failure or other complications. Herein, we report a 64 year-old man with dual coronary artery fistulas presenting with exertional chest pain. The fistulas were initially suspected on transesophageal echocardiogram because of abnormal flow with a mosaic pattern between the left anterior descending and main pulmonary arteries. Selective coronary angiogram confirmed the diagnosis and revealed fistulous connections from the proximal left anterior descending and ostial right coronary arteries to the main pulmonary artery. The patient became symptom-free after surgical closure of the fistulas.     

先天性冠状动脉瘘20例临床分析

目的 总结先天性冠状动脉瘘的临床特点及诊断、治疗方法.方法 回顾分析2001年9月至2006年7月我院收治通过选择性冠状动脉造影确诊的20例先天性冠状动脉瘘患者的临床资料.结果 冠状动脉瘘来自左冠状动脉12例(60%),包括左主干至肺动脉瘘2例和左前降支至肺动脉瘘10例;来自右冠状动脉7例(35%),包括4例至肺动脉瘘和3例至右心室瘘;1例(5%)来自双侧冠状动脉,也均瘘入肺动脉.主要表现有胸闷、胸痛的9例(45%),劳累性气促7例(35%),心悸4例(20%).心电图表现正常8例,左心室肥大3例,右心室肥大4例,有心肌缺血表现5例.8例行介入封堵治疗,6例行外科手术治疗,其余6例未做治疗 .20例患者随访1个月～5年,预后良好.结论 先天性冠状动脉瘘可经选择性冠状动脉造影确诊,手术治疗或介入治疗均安全、有效,远期效果良好.     

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。     

26例胸腔镜辅助下微创二尖瓣手术的临床观察

胸腔镜辅助下二尖瓣手术26例(胸腔镜组),同期常规正中开胸二尖瓣手术30例(常规组),两组均无死亡病例,两组术后呼吸机辅助时间、术前左房内径及左室射血分数差异无统计学意义,胸腔镜组主动脉阻断时间及体外循环时间较常规组长(P<0.05),而胸腔镜组术后24 h胸液量明显减少(P<0.05),术后ICU监护时间、住院时间明显缩短(P<0.05),且胸腔镜组术后左房内径及左室射血分数较常规组明显改善(P<0.05),术后肺动脉压力下降也较常规组明显(P<0.05)。