复杂型先天性心脏病大型主-肺动脉侧支血管的形态学特征

目的 探讨复杂型先天性心脏病(先心病)患儿大型主-肺动脉侧支血管(major aortopulmonary collateral arteries,MAPCAs)的形态学特征.方法 回顾性分析伴MAPCAs的复杂型先心病患儿的MAPCAs的影像学资料,总结MAPCAs发布规律、数量及形态学特征.本组51例伴MAPCAs的复杂型先心病患儿中肺动脉闭锁/室间隔缺损33例(64.71％),法洛四联症11例(21.57％),右心室双出口2例(3.92％),完全性大动脉转位1例(1.96％),完全性房室间隔缺损1例(1.96％),肺动脉狭窄l例(1.96％),肺动脉吊带1例(1.96％),肺动脉缺如l例(1.96％).另外,合并动脉导管未闭16例(31.37％).结果 51例患儿中存在中央共汇43例(84.31％),MPCAs共117支,每例1～6支,平均2.29支.MAPCAs起始直径2.0～18.0 mm,平均4.3mm.MAPCAs起源于胸降主动脉75支(64.10％),主动脉弓16支(13.68％),左锁骨下动脉12支(10.26％),右锁骨下动脉11支(9.40％),头臂干2支(1.71％),其他1支(0.85％).本组3支(2.56％) MAPCAs开口狭窄及狭窄后扩张,57支(48.72％) MAPCAs在进入肺脏前出现迂回、扭曲;8例(15.69％)患儿MAPCAs为惟一血源供应相应肺段.另有13支(11例,均为肺动脉闭锁/室间隔缺损)较粗大的MAPCAs可见分支,其分支为2～4支,多为2支.结论 MAPCAs多见于某些肺血减少型先心病,且分布较广泛,其起源、数量、大小、走行以及分支等方面变异较大,主要影响手术方式选择及疗效,了解MAPCAs的分布规律及形态学特征具有十分重要的临床意义.     

大型主—肺动脉侧支血管及其处置策略

大型主—肺动脉侧支血管(major aortopulmonary collateral arteries,MAPCAs)指起源于主动脉或其侧支,与外周肺动脉连接,或提供肺动脉血流至肺段或肺叶,而与中央肺动脉无连接的血管[1].MAPCAs多见于某些肺血减少型先天性心脏病(先心病),如肺动脉闭锁(29.7％)、法洛四联症(3.5％[2])、右心室双出口、完全性房室隔缺损和大动脉转位等,罕见发生于严重肺动脉狭窄者.纠治合并MAPCAs的复杂先心病时,正确处置MAPCAs至关重要.目前,国内、外有关MAPCAs的文献资料尚较少,现综述如下.     

法洛四联症合并异常冠状动脉的外科治疗

目的 总结法洛四联症(TOF)合并异常冠状动脉完全矫治手术的经验.方法 自1993年3月至2006年4月对22例TOF合并异常冠状动脉的患者施行完全矫治手术,其中经右室前壁小切口径路切除右心室流出道(RVOT)内肥厚的隔束、壁束以及纤维狭窄环,并修补室间隔缺损(VSD)5例;经右心房径路疏通RVOT、修补VSD17例,其中跨肺动脉瓣环补片加宽RVOT 7例,用自体心包外管道重建RVOT 3例,同种带瓣血管重建RVOT 1例,肺动脉前壁血管片反折缝合于RVOT上缘,外用带单瓣的牛心包补片缝合,将RVOT重建为外通道2例;其余患者均经右心房-肺动脉径路解除RVOT狭窄.结果 术后早期因冠状动脉损伤死亡1例.1例误将发自右冠状动脉(RCA)的附前降支当作增粗的圆锥支予以切断,导致停体外循环困难,将左乳内动脉与该血管吻合后停机成功,其余患者均顺利恢复.术后早期右心室-肺动脉压差(△P)平均为23.4mmHg,△P＞20mmHg者9例.随访18例,平均随访13.2个月,失访3例.△P＞20mmHg者7例,其余患者△P均＜20mmHg.结论 经右心房径路修补VSD并疏通RVOT使手术更加安全,RVOT的重建方式应根据异常冠状动脉的类型等具体解剖情况来决定.     

肺血减少型先天性心脏病肺血来源的探讨

肺血减少型先天性心脏病（先心病）包括法洛四联症（TOF）、肺动脉闭锁（PAA）及伴有肺动脉狭窄（PS）的心室双出口（DOV）、三尖瓣闭锁（TA）、完全性大动脉转位（TGA）、矫正性大动脉转位（CTGA）、单心室（sV）等复杂畸形。其肺血来源多样，不仅限于肺动脉，还有未闭动脉导管和体肺侧支，外科矫治困难。现回顾性探讨不同种疾病肺血来源的多样性及与肺动脉的关系，以增加对此类疾病的认识。     

合并室间隔缺损及粗大体肺侧支的肺动脉闭锁的选择性侧支融合术及粗大体肺侧支的再认识

目的探讨选择性侧支融合术或单源化治疗(unifocalization,UF)在室间隔缺损(VSD)及粗大体肺侧支(MAPCAs)的肺动脉闭锁(PA)患儿中的应用,并从组织病理学角度深入剖析不同解剖形态的MAPCAs。方法2017年6月至2018年12月,6例合并粗大体肺侧支及室间隔缺损的肺动脉闭锁(MAPCAs/VSD/PA)患儿实施选择性UF术加一期根治术,男4例,女2例;年龄6~96个月;体质量5.0~23.0 kg。术前均行心导管、选择性体肺侧支造影及心脏CTA检查,明确体肺侧支种类及解剖分布。对造影提示参与肺"气血交换"的粗大体肺侧支实行选择性UF,对不参与肺"血气交换"的功能性体肺侧支术中结扎或不处理。术中留取不同种类粗大体肺侧支的组织行病理检查,根据组织病理学结果明确侧支类型。结果围术期及随访期间无死亡。术前心导管、选择性侧支检查及术中组织病理学检查证实,参与肺"血气交换"的功能性MAPCAs具有肺动脉壁结构,属于弹性动脉;不参与肺"血气交换"的非功能性MAPCAs为小动脉壁结构,属于肌型动脉。结论术前可通过选择性MAPCAs造影检查明确参与肺"血气交换"功能性侧支,功能性侧支属于弹性动脉,具有肺动脉壁结构,术中对功能性侧支进行UF术可取得良好的手术效果。     

心脏外科手术与介入性心导管术镶嵌治疗小儿先天性心脏病

目的 报道23例介入性心导管术与心脏外科手术镶嵌治疗小儿先天性心脏病(CHD)的经验。方法外科术后镶嵌治疗：12例动脉导管未闭结扎术后残余分流及2例心脏术后留置室间隔缺损(VSD)的复杂CHD行经导管封堵术。2例法洛四联症(TOF)根治术后分支肺动脉狭窄分别行球囊血管成形术及支架置入术，1例肺动脉闭锁合并VSD根治术后出现体肺侧支血管(APCAs)破裂行介入栓塞止血术。外科术前介入治疗：5例室间隔完整的大血管转位(TCA)病例行大动脉调转术前行球囊房隔造口术(BAS)，1例有巨大APCAs的重症。TOF在根治术前予以侧支血管堵塞术。结果动脉导管未闭术后残余分流及复杂CHD残留VSD病例均封堵成功，分支肺动脉狭窄病例介入治疗后狭窄减轻或治愈；侧支血管破裂病例栓塞止血后出血停止。BAS病例介入术后低氧及酸中毒改善，大动脉调转术后3例治愈，2例死亡。TOF病例APCAs堵塞后成功行根治术。结论心脏外科手术与介入性心导管术镶嵌治疗是治疗小儿CHD术后残余分流及部分复杂疑难CHD的有效方法。     

喉罩在先天性心脏病伴气管狭窄患儿麻醉中的应用

先天性心脏病(先心病)伴气管狭窄,以往在先心病矫治术时气管狭窄不做处理,有些患儿会为此发生术后脱机困难;如果气管狭窄行一期矫治手术(去除狭窄段断端吻合术或补片扩大管径术等),手术创伤大,死亡率高[1],本文采用喉罩全麻下先心病矫治术联合纤支镜介入技术,检查评估先心病矫治术后气管狭窄改善情况,对中、重度气管狭窄患儿行纤支镜下球囊扩张、气管支架置入术等治疗,取得了很好     

肺动脉融合术治疗肺动脉闭锁合并大主肺侧支动脉

目的 总结肺动脉融合术治疗肺动脉闭锁、室间隔缺损合并大主肺侧支动脉的初步经验.方法 1999年12月至2007年6月完成肺动脉闭锁,室间隔缺损合并大主肺侧支动脉(PA/VSD/NAPCAs)的肺动脉融合术17例,其中男7例,女10例;年龄0.8～18岁,平均(6.0±5.6)岁;体重6.5～55.0 kg,平均(20.0±14.9)kg.术前固有肺动脉指数(PAJ)为42.0～366.1mm2/m2,平均(133.7±87.8)mm2/m2.手术入路包括正中剖胸10例,正中+左后外侧剖胸5例,正中+右后外侧剖胸2例.一期肺动脉融合及心内畸形矫治术12例,肺动脉融合及姑息性体肺分流术4例,肺动脉融合及姑息性右室流出道扩大1例.全组病儿术前造影发现大主肺侧支动脉共44支;术中融合29支,结扎5支.肺动脉闭锁矫治术及肺动脉融合术前介入栓堵3个侧支1例;肺动脉融合及体肺分流术后介入栓堵2个残留侧支1例.结果 全组术中转流174.3min,主动脉阻断88.7min.术后呼吸机辅助7.4d,术后ICU 11.8d.生存11例,平均新建肺动脉指数(TNPAI)249mm2/m2.6例死亡,其中2例TNPAI<200mm2/m2,死于肺血管发育不良,术后发生低心排出量综合征(低心排)和突发室性心律失常;1例因低心排无法脱离体外循环,尸检发现肺小动脉明显肌型化,管腔高度狭窄,证实术前局部肺段已有严重的肺高压;3例>450 mm2/m2分别死于严重肺部感染、渗出、急性肾功能衰竭,反复气道大量出血和顽固性室性心律失常.其他主要并发症为大脑皮层盲、膈肌麻痹和阴沟杆菌性肺炎肺出血各1例.结论 完全矫治+肺动脉融合是治疗肺动脉闭锁/室间隔缺损合并大主肺侧支动脉最根本的治疗手段,但手术操作复杂.由于同一病儿肺动脉病变的多样性,常见MAPCAs高压及狭窄并存.适应证选择和手术技巧的提高仍需不断的探索.     

改良主、肺动脉根部置换术治疗伴左室流出道梗阻的大动脉错位

目的 探讨一种新的主、肺动脉根部置换手术治疗合并室间隔缺损(VSD)、左室流出道梗阻(LVOTO)的大动脉错位(TGA).方法 4例伴LVOTO、VSD的TGA患者及1例伴LVOTO、VSD的右心室双流出口患者,被采用改良的Nikaidoh技术将主动脉和肺动脉根部完整互换移植、冠状动脉移植以及双心室流出道重建;其中2例房室异常连接患者被同期施行Senning手术.结果 所有患者手术均获成功,术后恢复良好.术后平均随访5.40个月,生长发育好.超声心动图检查提示心室功能良好,主动脉瓣无反流;2例患者肺动脉瓣有少量反流.结论 保留半月瓣的主、肺动脉根部置换术治疗伴LVOTO的复杂型TGA患者,不仅可获得解剖根治,同时解决了右心室流出道的非生长性问题,改进的冠状动脉再植技术扩大了Nikaidoh手术的适应范围,并获得很好的近期疗效.但其远期疗效仍需更大组的手术例数及更长期的随访来验证.     

肺动脉后壁扩大法矫治合并左肺动脉狭窄的法洛四联症

目的总结采用肺动脉后壁扩大法矫治合并左肺动脉狭窄的法洛四联症(TOF)的手术技术要点及效果。方法 2008年1月至2014年8月共1 142例患者在我院行TOF矫治术,合并左肺动脉严重狭窄的患者44例。其中21例采用肺动脉后壁扩大矫治左肺动脉狭窄的手术策略。年龄1个月至11岁,平均(22.8±33.9)个月,体重3.8~29.0(9.1±5.3)kg。结果 1例术中死亡,20例长期生存。4例术后出现单侧右肺渗出。心脏彩色超声心动图随访3~72个月,14例左肺动脉处无压差,6例残留压差(32.8±12.7)mm Hg。1例压差逐渐增大行介入球囊扩张。结论肺动脉后壁扩大法可有效矫治TOF合并的左肺动脉狭窄,扩大后补片,血流方向更顺畅。切断动脉导管可减少牵拉导致的肺动脉扭曲。同时该术式增加新建肺动脉的自体组织,有更好的生长潜力。     

A case of coronary artery-pulmonary artery fistula in tetralogy of Fallot with pulmonary atresia and major aortopulmonary collateral arteries (MAPCA)]

A case of four year old girl with tetralogy of Fallot, pulmonary atresia, major aortopulmonary collateral arteries and coronary artery to pulmonary artery fistula is reported. The patient was misdiagnosed as truncus arteriosus, pulmonary stenosis and major aortopulmonary collateral arteries (MAPCA) preoperatively. The ligation of MAPCAs was carried out through left thoracotomy as an initial procedure, because we believed that the area fed by MAPCAs had dual blood supply. However, the patient demonstrated pulmonary infarction post operatively. This complication forced us to postpone an intracardiac repair. At the time of intracardiac repair, after mentioned diagnosis was established and a Rastelli type operation and ligation of coronary-pulmonary artery fistula were carried out. A 18 mm Carpentier-Edwards valved conduit was used as an extracardiac conduit. In conclusion: 1) We experienced a rare association of coronary artery fistula in TOF with PA. 2) The MAPCAs functioning as a sole source of blood-supply to the lung should be precisely diagnosed preoperatively and uniforcalized before an intracardiac repair.     

Transpulmonary Stenting of Both Pulmonary Arteries with a Surgical Access through Redo Sternotomy

Abstract   We report the case of a four-year-old boy with pulmonary atresia (PA) and ventricular septal defect (VSD) associated with MAPCAs who had a complete repair two times. He developed 21 months later stenosis at the origin of both pulmonary arteries. The conventional interventional catheterization approaches were not available due to femoral vein thrombosis and severe transient complete atrioventricular block due to the manipulations to catheterize the left pulmonary artery. The stenoses were treated by implant of stents using a surgical access to the proximal portion of a Contegra valved conduit (Medtronic, Inc., Minneapolis, MN, USA) by sternotomy.     

分期全腔静脉-肺动脉连接术治疗复杂先天性心脏病

目的 总结分期全腔静脉.肺动脉连接术(TCPC)治疗复杂先天性心脏病的经验.方法 1998年6月至2008年3月,22例先天性心脏病复杂畸形患者接受分期TCPC.本组中单心室合并肺动脉狄窄9例,合并肺动脉闭锁3例;完全性大动脉转位,十字交叉心,肺动脉瓣狭窄1例;完全性房室通道,左心窒发育不良,肺动脉闭锁,房室瓣少-中量反流1例;完全性房室通道,动脉导管未闭,肺动脉狭窄,双向Glenn术后伴吻合口狭窄房室瓣大量反流1例;镜面右位心,功能性单心室,肺动脉闭锁,动脉导管未闭,体肺侧支,双侧双向Glenn术后2年,右下肺动-静脉瘘1例;三尖瓣闭锁并肺动脉狭窄4例;三尖瓣闭锁伴肺动脉闭锁,动脉导管未闭1例;镜面右位心,右心室双出口,窒间隔缺损,左心室发育小良,功能性单心房,肺动脉瓣狭窄,三尖瓣少量反流,粗大体肺侧支1例.第一期体肺分流术后,二期双向Glenn术5例,第一期单侧或双侧双向Glenn手术17例.行双向Glenn手术的年龄为(5.9±4.4)岁,Glenn术前肺动脉压为17～20 mm Hg(1 mm Hg=0.133 kPa);房室瓣膜关闭不全3例;一期手术前Nakata指数小于200 mm2/m2者4例.接受TCPC术平均年龄为(9.6±4.9)岁,与双向Glenn术间隔时间(3.7 ±1.2)年,术前经上腔静脉置管测得肺动脉压力均小于15 mm Hg.全组22例均采用心外管道TCPC,房窜瓣成形1例.结果 全组住院死亡1例,病死率为4.5%.死亡病例为单心室,肺动脉闭锁,第一期体肺分流术后,二期左肺动脉成形双向Glenn术后5年第三期行TCPC,术后左肺反复多次大出血死亡.余患者均顺利出院,术后中心静脉压12～18 mm Hg.出院前经皮测血氧饱和度为90%～96%,活动量明显增加,发绀及气促症状消失,心功能均为NYHA分级Ⅰ～Ⅱ级.结论 对于高危患者根据病情选择分期TCPC,可以使更多有高危因素不能行Fontan手术的患者得到救治的机会,扩大手术适应证,并取得满意疗效.     

Decision-making in unoperated adults with congenital heart disease: a difficult task

The authors present a 32-year-old lady with a diagnosis of VSD, pulmonary atresia and multiple aortopulmonary collateral arteries (MAPCAs), who was palliated successfully by stent implantation. This interventional procedure is discussed as well as the decision-making process in adult patients with unoperated congenital heart disease.     

Single-stage unifocalization and correction with median sternotomy in complex pulmonary atresia

OBJECTIVE: This study assessed the surgical and post-operative outcome of single-stage complete unifocalization and repair procedure in patients with complex pulmonary atresia. METHODS: From 1999 to 2001, we performed complete unifocalization and correction in 10 patients with complex pulmonary atresia. Their ages ranged from 10 months to 17 years. All patients were evaluated with pulmonary angiography and divided into two groups according to the development of native pulmonary arteries. Group I patients had hypoplastic pulmonary arteries and MAPCAs and Group II patients had only MAPCAs without native pulmonary arteries. With median sternotomy, all MAPCAs were prepared and anastomozed to native pulmonary arteries in group I patients or on a pericardial roll in group II patients without using cardiopulmonary bypass. Right ventricle to pulmonary arterial continuity was established with a valved conduit under CPB. VSD was closed in two patients. RESULTS: Eight patients had complete repair without VSD closure. They were followed periodically with pulmonary angiography. Two patients developed congestive heart failure. One of them was reoperated and VSD was closed. The other patient died because of untractable congestive heart failure. The decision for VSD closure was made in two patients due to suitable pulmonary arterial vascular tree. However, one of them had to be reoperated and VSD patch was removed. This patient died because of sepsis on the postoperative 26th day. We are following the rest of the patients with echocardiography and pulmonary angiography. CONCLUSION: Single stage complete unifocalization and repair should be the treatment of choice in patients with complex pulmonary atresia. This procedure provides a significant development in neopulmonary arterial system. However, the accurate criterias for VSD closure are still controversial. After the operation, these patients had to be followed closely with echocardiography and pulmonary angiography because of the absolute risk of congestive heart failure in patients with VSD left open.     

Coronary to pulmonary artery collaterals in patients with pulmonary atresia and ventricular septal defect

BACKGROUND: The frequency, distribution, and surgical importance of coronary artery to pulmonary artery (CAPA) collaterals have not been established. The aim of this study was to establish prevalence, anatomical pattern, and significance of CAPA in patients with pulmonary atresia and ventricular septal defect (PA/VSD). METHODS: We reviewed cardiac catheterization and operative data of 87 consecutive, unselected patients who underwent one-stage complete unifocalization for PA/VSD and assessed major systemic to pulmonary collaterals from July 1992 to June 1998. RESULTS: CAPA collaterals were diagnosed in 9 of 87 patients (10%). The collaterals originated from the left coronary artery system in 7 patients and the right in 2. Collaterals from the left coronary system arose from the left main coronary artery in 3 patients and the circumflex in 4. All collaterals joined the central pulmonary artery, which bifurcated and supplied both lungs. One collateral from the right coronary system joined the stump of the main pulmonary artery and the other gave origin to a true left pulmonary artery, which was the sole supply to 75% of the left lung. Coronary artery enlargement was seen in 2 patients only. No patient had evidence of myocardial ischemia. Coronary collaterals comprised a dual source of pulmonary blood flow in all but 1 patient. During unifocalization, the CAPA collaterals were ligated at its origin in all cases, and the collateral from the right coronary to the left pulmonary artery was unifocalized. CONCLUSIONS: The prevalence of CAPA collaterals in patients with PA/VSD is approximately 10%. The diagnosis may be missed without appropriate angiograms. We recommend selective ascending aortogram or selective coronary angiogram in all patients.     

Median sternotomy single stage complete unifocalization for pulmonary atresia, major aorto-pulmonary collateral arteries and VSD-early experience.

OBJECTIVE: It is a prospective study to assess the results of median sternotomy, single stage complete unifocalization and repair for ventricular septal defect (VSD), pulmonary atresia and major aorto pulmonary collateral arteries (MAPCAs). METHODS: From June 97 to August 98, 20 patients were treated with single stage complete unifocalization and repair. Their ages ranged from 6 months to 11 years. Through median sternotomy, all MAPCAs were dissected and looped. On cardiopulmonary bypass, MAPCAs were anastomosed to native pulmonary arteries (PAs) or to MAPCAs. VSD was closed if possible and RV to PA continuity was established with a homograft conduit. If complete repair was not suitable, central shunt was done from ascending aorta to reconstructed PA with a polytetrafluroethylene graft. The patients were divided into three groups according to the arborization pattern in the lungs. Group 1 had well formed native PAs with MAPCAs, group 2 had hypoplastic PAs with MAPCAs and group 3 had only MAPCAs. RESULTS: Twenty patients had 21 procedures. All MAPCAs were unifocalized with tissue-to-tissue anastomosis for future growth, except one in whom polytetrafluroethylene tube graft was used to attain the confluence. In group 1, all seven patients had complete unifocalization and repair. In group 2, four patients had RV to PA conduit and two patients had central shunt. In group 3, three patients had complete repair, three patients had RV to PA conduit and one patient had central shunt. There were three deaths, two in group 2 and one in group 3. The first patient died due to a wrong decision to close the VSD, the second patient died due to missed large MAPCA in preoperative angio and the third patient was a 7-year-old boy who died with irreversible pulmonary vascular changes due to unprotected MAPCAs. CONCLUSIONS: To conclude, complete repair/RV-PA conduit/central shunt should be done according to the size of the total pulmonary vasculature in patients with group 1, 2 and 3 with protected PAs/MAPCAs and in hypoplastic or absent PAs with unprotected MAPCAs (less than 1 year) and protected MAPCAs. We are yet to determine the surgical procedure to be performed in hypoplastic/absent PAs with unprotected MAPCAs more than 1 year. It is very essential to delineate all the MAPCAs up to the level of the diaphragm preoperatively.     

A case of pulmonary arteriovenous fistula with mitral stenosis

The patient was a 61-year-old female, complaining of cyanosis, dyspnea and shortness of breath on exertion. She was diagnosed as having a pulmonary arteriovenous fistula (PAVF) in combination with mitral stenosis. The fistula was located in the left lower lobe and a right-left shunt of 28.7% was detected. Cardiac catheterization showed a pulmonary artery pressure of 44/22 mmHg (mean pressure, 31 mmHg). By occluding the PAVF using a balloon catheter, PaO2 increased from 47 mmHg to 88 mmHg. The mitral stenosis of this patient was though to be a mild form, and PAVF seemed to be responsible for symptoms. Since left lobectomy together with mitral valve replacement was considered to have a high risk, left lower lobectomy was performed initially. Thereafter mitral valve replacement was done successfully. Separate operations for PAVF and mitral stenosis are likely to be beneficial in patients with mitral stenosis associated with moderate pulmonary hypertension.