“一站式”Hybrid技术治疗法洛四联症合并体肺动脉侧支血管形成

目的总结应用"一站式"Hybrid技术治疗合并体肺动脉侧支的法洛四联症的临床效果。方法 2009年5月-10月采用"一站式"Hybrid技术治疗合并体肺侧支的法洛四联症患者2例。男、女各1例,年龄分别为39、32岁,均因全身渐进性青紫及反复咯血入院。心导管检查显示,病例1右支气管动脉、右甲状颈干动脉分别与右肺动脉形成粗大侧支循环,左胸廓内动脉与左肺动脉形成粗大侧支循环;病例2左支气管动脉与左肺动脉形成粗大侧支循环。结果 2例患者共5支体肺动脉侧支均成功栓堵,术中及术后无严重缺氧以及肺叶坏死,一期根治手术均成功施行,超声心动图检查无室缺残余瘘发生。患者术后获随访6个月均能正常生活。结论应用"一站式"Hybrid技术治疗合并较大体肺侧支的法洛四联症可降低手术难度,减轻手术创伤,安全有效。     

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.     

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.     

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。     

左肺反向循环动物模型的建立

目的探讨反向肺循环动物模型的建立方法及其可行性。方法选正常犬作为实验对象，利用牛颈静脉作为连接血管材料，建立左肺反向循环模型。于反向循环术前、术后2h、2周时测定血流动力参数和动脉血气，用超声流量仪测定左肺动脉流量。动物饲养到2周时行心血管造影检查，观察反向循环肺灌注情况，动物处死后取部分肺组织行病理学检查。结果正常犬行左肺反向循环术后2h、术后2周血流动力学无明显改变，并能维持正常的动脉血气，反向循环后左肺动脉流量无减少，心导管造影右侧肺动脉及其分支显影清晰，左侧牛颈静脉及肺静脉系统亦显影清晰，吻合口通畅。大体观察左、右肺无差别，组织病理学检查左、右肺亦无明显差异。结论左肺反向循环具有可行性，可维持良好的血流动力学、肺的正常结构和通气交换功能。     

肺动脉闭锁合并室间隔缺损及粗大体肺侧支外科治疗104例临床分析

目的探讨采用优化的肺血管单源化策略治疗肺动脉闭锁合并室间隔缺损及粗大体肺侧支患儿的早、中期结果。方法回顾性分析2017年1月至2022年9月在广州医科大学附属广州市妇女儿童医疗中心心脏中心接受手术治疗的104例肺动脉闭锁合并室间隔缺损及粗大体肺侧支患儿的临床资料、手术记录及随访结果。男55例, 女49例, 年龄[M(IQR)]33.9(84.0)个月(范围:0.5～209.6个月)。解剖分型:B型89例, C型15例;粗大体肺侧支4.2(3.0)支(范围:1～8支)。采用Kaplan-Meier法绘制生存曲线。结果一期手术中, 50例接受完全根治术, 12例接受部分根治术, 32例接受姑息性右心室-肺动脉连接术, 10例选择了体肺分流。一期完全根治率为48.1%(50/104), 术后早期病死率为9.6%(10/104)。二期手术中, 14例获得完全根治, 4例获得部分根治, 无早期死亡。总体根治率为61.5%(64/104)。两期手术间隔时间为19(10)个月(范围:9～48个月)。完全根治术后右心室/左心室收缩峰压比值为0.63±0.16(范围:0.36～1.00)。全组患儿一期手...     

重症法洛四联症矫正术前应用体肺侧支血管栓塞术的价值

目的：评价体肺侧支血管栓塞术在重症法洛四联症矫正术中的应用价值。方法：经心血管造影证实诊断的5例成年重症法洛四联症病人，在施行外科心内畸形矫正术当天先送导管室，行体肺侧支血管栓塞术。结果：造影发现体肺侧支血管16支，对其中的12支（75％）侧支血管进行了成功栓塞，共用弹簧栓子18枚。外科术中术野显露较好，回血量不多，体外循环灌注压稳定。术后4例痊愈，1例因左心室重度发育不良而死于低心输出量综合征。结论：对伴有丰富侧支血管形成的复杂紫绀性先天性心脏畸形病人，在外科矫正术前通过造影指导下栓堵体肺侧支血管，可简化手术过程，减少因手术野限制结扎体肺侧支血管的困难，提高手术成功率。     

采用双冠状动脉导丝法建立先天性心脏病介入治疗复杂路径轨道

目的探讨以双冠状动脉导丝法建立先天性心脏病(简称先心病)介入治疗复杂路径轨道的价值。方法回顾性分析12例应用双冠状动脉导丝法建立介入治疗轨道的先心病患儿的临床资料,其中肺动脉闭锁伴室间隔缺损(PA/VSD)矫治术后肺动脉狭窄5例,法洛四联症(TOF)矫治术后肺动脉狭窄1例,大动脉转位(TGA)术后肺动脉狭窄1例,粗大体肺侧支(MAPCAs)3例,复杂型肺动静脉瘘1例及左冠状动脉回旋支-右心房瘘1例;分析先心病介入治疗复杂路径特点。结果采用双冠状动脉导丝均成功建立输送轨道。对PA/VSD矫治术后肺动脉狭窄、TOF术后肺动脉狭窄、TGA术后肺动脉狭窄患儿成功进行球囊扩张,对MAPCAs、复杂型肺动静脉瘘及左冠状动脉回旋支-右心房瘘患儿均封堵成功,未出现瓣膜损伤、血管损伤、心包填塞及死亡等严重并发症。结论以双冠状动脉导丝法建立先心病介入治疗复杂路径轨道方法安全、有效,可调控性强,能提供足够支撑力通过纡曲段,有利于提高介入治疗成功率,值得临床推广应用。     

主动脉弓离断并法洛四联症、右锁骨下动脉及降主动脉侧支起源1例

病儿女,12岁。自幼发现心脏杂音,全身发绀,有蹲踞现象。查体血压右上肢90/70mmHg(1mmHg=0.133kPa),左上肢100/70mmHg,右下肢110/70mmHg,左下肢110/80mmHg。口唇及颜面发绀,心率80次/min,律齐,胸骨左缘3、4肋间可闻及4/VI级喷射性收缩期杂音,杵状指(趾)。心电图示窦性心律,电轴右偏(109°),右室肥厚。X线胸片示心胸比率0.53,双肺血稀疏,心腰凹,右室增大,双上腔静脉可疑。心脏彩色超声示右心房、室增大,左心腔内径相对较小,主肺动脉及分支内径正常。左室长轴及大动脉短轴切面显示主动脉大部分发自右室,主动脉在右前,肺动脉在左后;右室流出道8mm;室间隔与主动脉前壁回声连续中断23mm;左上腔静脉永存。动脉血气分析PCO_235mmHg,PO_252mmHg,SaO_20.87。经皮四肢末梢血氧饱和度左上肢0.87,右上肢0.86,双下肢均为0.87。左、右心血管造影见两大动脉均起自右室,右室流出道狭窄,膜部室间隔缺损,主动脉弓于左锁骨下动脉后中断,右锁骨下动脉及降主动脉由主动脉侧支形成(图1～8)。图1右心室造影示右室流出道狭窄图2、3右心室造影示升主动脉、主肺动脉同时显影,膜部室间隔缺损,右室流出道狭窄,肺动脉发育佳图4猪尾心导管经右股动脉(测压证实)到达侧支形成的降主动脉起始处造影,见造影剂向下快速充盈图5心导管经右桡动脉、右锁骨下动脉(测压证实)进入降主动脉造影,见造影剂向下快速充盈图6心导管经右桡动脉、右锁骨下动脉到达侧支形成右锁骨下动脉和降主动脉的共汇处造影,见造影剂向两处快速充盈图7心导管经左桡动脉、左锁骨下动脉进入升主动脉造影,见左、右颈总动脉和左锁骨下动脉均显影,右锁骨下动脉未显影,左锁骨下动脉以后主动脉弓中断图8图7的下一时相,见大量侧支形成右锁骨下动脉及降主动脉2004年8月在全麻低温体外循环下行法洛四联症根治术。术中见主动脉∶主肺动脉为2∶1,右室扩大,右室流出道肌性狭窄,肺动脉瓣环狭窄,直径0.5cm。肺动脉瓣二瓣化畸形,主肺动脉及左、右肺动脉发育良好。嵴下型室间隔缺损3.0cm×2.5cm大小。主动脉骑跨70%以上,二尖瓣与主动脉瓣有纤维连续。左上腔静脉永存。疏通右室流出道,血管片补片关闭室间隔缺损,垫自体心包的涤纶片跨肺动脉瓣环加宽右室流出道。考虑病儿主动脉系侧支循环丰富,对主动脉中断未处理。术终测上、下肢动脉平均压差仅10mmHg。病儿术后恢复顺利,术后10d痊愈出院。现已随访28个月,病儿恢复正常生活和学习。讨论主动脉弓离断罕见,病死率较高,在先心病婴儿尸检中,发现率为1.4%〔1〕。室间隔缺损和动脉导管未闭是最常见的合并畸形,亦可合并永存共同动脉干、主肺动脉间隔缺损、大动脉转位、右肺动脉起源异常、右肺动脉闭锁和左冠状动脉异常起源于右肺动脉等。心血管造影是术前确诊、分型及鉴别诊断的重要方法。MRI和UFCT也是诊断本症的重要手段。外科手术可一期根治,也可分二期进行,先恢复主动脉弓连续性、再矫治合并畸形。目前倾向一期手术〔1-4〕。     

肺保护液对先天性心脏病伴重度肺动脉高压患者的肺保护作用

我科自 2 0 0 0年 11月～ 2 0 0 2年 1月在 11例先天性心脏病伴重度肺动脉高压患者手术中 ,全部采用肺动脉灌注肺保护液进行肺保护 ,获得了较为满意的临床效果。1　临床资料与方法1.1　一般资料　本组共 11例 ,男 5例 ,女 6例 ;年龄 5～ 12岁 ,平均年龄 7.3± 3.6岁 ;体重 13～ 4 0 kg,平均体重 2 4 .7±9.7kg。病种 :室间隔缺损 (VSD) 6例 ,原发孔型房间隔缺损(ASD) 2例 ,室间隔缺损 +房间隔缺损 (VSD+ASD) 1例 ,右心室双出口 +动脉导管未闭 (DORV+PDA) 1例 ,完全性肺静脉异位引流 (TAPVC) 1例。 10例行右心导管检查 ,肺动脉压11…     

肺动脉闭锁合并室间隔缺损及大的体肺动脉侧支患者肺循环的解剖分析和临床意义

目的 分析肺动脉闭锁合并室间隔缺损和大的体肺动脉侧支的患者肺循环的病理解剖特点,并探讨其临床意义.方法 回顾性分析2002年4月至2010年6月33例肺动脉闭锁合并室间隔缺损和大的体肺动脉侧支的患者,男性21例,女性12例,年龄11个月～29岁.根据1999年国际儿童心脏外科数据和命名会议的分型标准,B型22例,C型11例.29例(87.9%)存在自身肺动脉,其中发育较好的6例(18.2%),发育不良或左右肺动脉无汇合的23例(69.7%).4例(12.1%)自身肺动脉完全缺如.其中31例进行了33次手术治疗,包括主动脉-肺动脉分流手术8例,一期单源化手术2例,矫治手术23例.结果 分流手术和一期单源化手术的患者术后动脉氧饱和度83%～90%.矫治手术早期死亡1例,死亡原因为多脏器功能衰竭;低心排血量综合征4例,低氧血症(氧合指数<150 mmHg,1 mmHg=0.133 kPa)3例.16例矫治手术患者随访超过1年,右心室压41～99 mmHg;肺动脉瓣中度反流2例;射血分数>50%者14例,<50%者2例.结论 肺动脉闭锁合并室间隔缺损和大的体肺动脉侧支应根据肺血管的解剖特点采取个体化治疗的方案,存在中央肺动脉、左右肺动脉有汇合、体肺动脉侧支与肺动脉之间存在肺内交通是决定一期单源化和心内矫治手术的关键.

Abstract：

Objectives To analyze the anatomy features of the pulmonary circuits in the patients with pulmonary atresia (PA) with ventricular septal defect (VSD) and major aortopulmonary collateral arteries (MAPCA), and discuss the clinical significance. Methods From April 2002 to June 2010, the anatomy features of pulmonary circuits in 33 patients with PA/VSD/MAPCA were examined and analyzed. There were 21 male and 12 female patients. The age ranged from 11 months to 29 years. The anatomic types of PA/VSD included group B for 22 cases, group C for 11 cases. Thirty-one patients of them underwent 33 operative procedures. The operations included aorta-pulmonary shunt in 8 cases, one stage unifocalization with VSD open in 2 cases, complete repair in 23 cases. Results Twenty-nine (87.9%) patients had native pulmonary arteries, 6 of them were normal size and 23 were hypoplastic size. Four patients (12. 1%) had no native pulmonary arteries. The postoperative oxygen saturation of the patients undergone shunt and one stage unifocalization was increased to 83% to 90%. There was one early death after complete repair because of multiorgan function failure. There were 4 cases of severe low cardiac output and 3 cases of respiratory function failure. Sixteen patients after complete repair were followed up more than one year. The postoperative right ventricular pressure was 41 to 99 mmHg (1 mmHg =0. 133 kPa). The ejection fraction value was more than 50% in 14 patients and less than 50% in 2 patients. Two patients had medium pulmonary insufficiency. Conclusions An individualized approach based on the anatomy of the pulmonary circuits permits achievement in the patients with PA/VSD/MAPCA. The surgical strategy for PA/VSD/MAPCA mainly depends on the anatomy features of native pulmonary arteries, confluent pulmonary arteries and MAPCA.     

Increasing experience with integrated approach to pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.

OBJECTIVES: To validate the preliminary surgical results of 'integrated approach' to pulmonary atresia, ventricular septal defect (VSD), and multiple aortopulmonary collateral arteries by retrospective analysis of our center experience. METHODS: Between 01/94 and 03/02, 37 patients aged 22 days to 13 years underwent surgery for pulmonary atresia, VSD, and multiple aortopulmonary collaterals. Case selection was based on preoperative calculation of total neopulmonary arterial index (TNPAI), pulmonary arterial index (PAI), and pulmonary arteries-to-collateral arteries lung segment perfusion ratio (S(pa):S(ca)). The decision for a possible VSD closure during one-stage procedures was based on an intraoperative pulmonary flow study. Twenty-five patients with a TNPAI equal to or greater than 150 mm(2)/m(2) underwent primary unifocalization, irrespective of PAI and S(pa):S(ca). Conversely, 12 patients with a TNPAI less than 150 mm(2)/m(2) and hypoplastic (PAI less than 100 mm(2)/m(2)) dominant (S(pa):S(ca) greater than 1) pulmonary arteries received a first-stage right ventricular outflow tract reconstruction, followed by unifocalization and repair (i.e. VSD closure) in nine cases. RESULTS: Among 34 patients who received total unifocalization, the overall repairability rate was 85% (first instance repairs: n=27; delayed VSD closure: n=2; 95% confidence interval, CI: 73-97%), with a survival rate at 7 years of 81%. Repaired survivors (n=26) are asymptomatic (n=22) or mildly symptomatic (n=4) at a follow-up interval of 43+/-28 months, with a 0.48+/-0.2 mean haemodynamic right ventricular/left ventricular pressure ratio, whereas palliated ones are waiting for either repair (n=3) or catheter study (n=2). Analysis of results has shown the following: (1) 100% (34/34 cases) feasibility of one-stage unifocalization in patients with a preoperative TNPAI equal to or greater than 150 mm(2)/m(2), whereas combined repairability rate was 79% only (95% CI: 65-93%); (2) 100% (12/12 cases) fulfillment of criteria for second-stage repairability (acquired TNPAI greater than 150 mm(2)/m(2)) in all patients treated with right ventricular outflow tract reconstruction; and (3) 93% (95% CI: 83-100%) overall accuracy of intraoperative flow study in predicting either postrepair mean pulmonary arterial pressure (VSD closed: n=23) or balanced pulmonary to systemic blood flow ratio (VSD left open: n=4). CONCLUSIONS: Increasing experience with 'integrated approach' to pulmonary atresia, VSD, and multiple aortopulmonaty collaterals has confirmed the preliminary results of our surgical series. The pulmonary flow study remains the most accurate intraoperative test for successful management of VSD during unifocalization procedures     

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 complex case of congenital cardiac anomaly: pulmonary atresia and ventricular septal defect associated with major aortopulmonary collaterals

Pulmonary atresia and ventricular septal defect associated with major aortopulmonary collaterals (MAPCAs) is a rare, complex, and heterogeneous congenital cardiac anomaly. The majority of untreated patients present with severe congestive heart failure and respiratory distress in the first decade of life. We describe a 15-year-old cyanotic boy, both of whose pulmonary arteries arise from the arcus aorta via patent ductus arteriosus. In addition to this anomaly, the patient has MAPCAs originating from the descending aorta that perfuse the right upper lobe of the lung, a persistent superior vena cava, an aberrant right subclavian artery, pulmonary atresia, and ventricular septal defect.     

Primary repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals: a useful approach

BACKGROUND: The ultimate goal of surgical therapy for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries is to create unobstructed and separate in series pulmonary and systemic circuits. Our preference has been a 1-stage complete unifocalization technique, avoiding collateral anastomosis with either the native pulmonary arteries or other aortopulmonary collateral vessels. METHODS AND RESULTS: Since 1998, 5 patients (median age 29.6 months) with pulmonary atresia, ventricular septal defect, and major aortopulmonary collateral arteries have undergone surgical correction, consisting of (1) exclusion of a descending thoracic aortic segment from which all major aortopulmonary collateral arteries originate, and (2) connection of this aortic segment to the native pulmonary artery using an interposition polytetrafluoroethylene conduit. The ventricular septal defect was closed in all patients, and the right ventricle was connected to the unifocalized pulmonary artery with a valved conduit. All patients survived the operation. Two patients required reexploration for postoperative bleeding. One patient remained on mechanical ventilation for 17 days due to a pulmonary infection. During follow-up (12-21 months), no patient required additional interventions. The postoperative right ventricular/left ventricular pressure ratio was 0.55 median. No significant stenosis within the reconstructed pulmonary circuit was identified. All patients remain free of symptoms, requiring no medications. CONCLUSION: Intracardiac repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries can be accomplished by a midline 1-stage repair including complete unifocalization of all pulmonary blood supply without individual collateral anastomosis in selected patients. This approach offers a convenient and satisfactory surgical option.     

Surgical creation of aortopulmonary window in selected patients with pulmonary atresia with poorly developed aortopulmonary collaterals and hypoplastic pulmonary arteries

OBJECTIVE: The morphologic characteristics of the pulmonary circulation vary widely in patients with pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals. Although we favor single-stage unifocalization and complete repair as the procedure of choice, a subgroup of patients who meet specific criteria have been treated with initial surgical creation of an aortopulmonary window. METHODS: Eighteen patients who were considered unsuitable candidates for single-stage repair underwent surgical creation of an aortopulmonary window. Selection criteria included the following: (1) presence of centrally confluent true pulmonary arteries 1.0 to 2.5 mm in diameter, with a well-developed peripheral arborization pattern; (2) multiple small aortopulmonary collateral vessels, most of which communicated with the true pulmonary arterial system; and (3) the presence of marked cyanosis. RESULTS: There were no early deaths, and the 2 late deaths were both unrelated to the procedure. Follow-up angiography (n = 17) demonstrated good growth of true pulmonary arteries in 15 of 17 cases (88%). Mean pulmonary artery diameter increased from 1.76 mm to 3.45 mm. Subsequent operations have been performed in 15 of 18 cases (83%). Intracardiac repair with or without additional unifocalization was achieved in 8 of these 15 (53%). Seven patients (47%) have undergone staged unifocalization as the next procedure; of these, 3 were able to have intracardiac repair. Thus 11 of the 15 patients who have undergone second operations (73%) have had complete intracardiac repair. CONCLUSIONS: The initial surgical creation of an aortopulmonary window in carefully selected patients can increase the size of the true pulmonary arteries, making these patients better candidates for eventual intracardiac repair. The procedure should be avoided in patients with pulmonary overcirculation, a predominance of isolated supply collaterals, or true pulmonary arteries larger than 2.5 mm in diameter, and it is not applicable without a true pulmonary artery central confluence.     

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.     

Surgical treatment of pulmonary atresia with ventricular septal defect

Pulmonary atresia with ventricular septal defect (PAVSD) is a complex cardiopathy represented by a complete obstruction between the right ventricle outflow and the pulmonary trunk associated with a ventricular septal defect (VSD) and major aortopulmonary collaterals (MAPCA). The goal of the unifocalization in the PAVSD is to prepare the pulmonary tree for the complete repair by connecting the MAPCAs to the central pulmonary arteries that should be enlarged. After that we can made the VSD or other intracardiac repair. This is a retrospective study on 31 patients. We report our results discussing the PAVSD classification and the strategy of the complete repair in comparison with other reported results.     

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.