非体外循环下肺动脉瓣切开术初期治疗室间隔完整型肺动脉闭锁的单中心临床分析

目的评价非体外循环下肺动脉瓣切开术在室间隔完整型肺动脉闭锁(PA/IVS)初期手术治疗中的疗效。方法回顾性分析2013年1月至2019年9月于我院行非体外循环下右心室减压术的61例PA/IVS患儿的临床资料,男37例、女24例,年龄29.7(2.0~86.0)d,体重4.1(2.5~6.9)kg。其中39例行非体外循环下肺动脉瓣切开术(直视切开组),22例行经右心室肺动脉瓣穿刺球囊扩张成形术(镶嵌治疗组)。比较两组患儿术后死亡率、早期再干预率以及终期手术情况等。结果全组共2例死亡,死亡率3.3%(2/61),直视切开组和镶嵌治疗组各死亡1例,两组死亡率差异无统计学意义(2.6%vs.4.5%,P=0.68)。两组在早期再干预率(5.3%vs.19.0%,P=0.09)、气管插管时间[(56.0±25.9)h vs.(62.0±28.9)h,P=0.41]、ICU滞留时间[(4.7±2.9)d vs.(5.5±2.2)d,P=0.23]、住院时间[(3.9±0.9)d vs.(4.3±1.1)d,P=0.38]等方面差异无统计学意义。随访时间45.3(4.0~84.0)个月,共5例失访。随访期间,直视切开组17例患者无需再手术,13例已完成终期手术,镶嵌治疗组7例患者无需再手术,8例完成终期手术,两组心功能分级(NYHA)均为Ⅰ~Ⅱ级。结论非体外循环下肺动脉瓣切开术与镶嵌治疗相比同样具有操作简便、手术时间短、手术成功率高等特点,且因更经济实惠及再干预率相对偏低,可能更易于临床推广应用。     

室间隔完整型肺动脉闭锁的右心室减压策略

目的 总结室间隔完整型肺动脉闭锁(PA/IVS)右心室减压的经验与教训,反思右心室减压策略.方法 回顾性分析2015年3月至2019年12月于我院行右心室减压手术的12例PA/IVS患儿的临床资料,其中男10例、女2例,手术时中位年龄5(1～627)d.对减压后肺动脉瓣跨瓣压差与三尖瓣Z值变化进行相关性分析.结果 术后...     

室间隔完整型肺动脉闭锁的外科治疗策略及中期随访分析

目的总结室间隔完整型肺动脉闭锁(pulmonary atresia with intact ventricular septum,PA/IVS)的个体化外科治疗策略和中期随访结果。方法回顾性分析2008年1月至2018年12月我院收治151例PA/IVS患儿的临床资料。将患儿分为两组:一期根治组26例,其中男17例、女9例,平均年龄(14.7±13.2)个月;分期手术组125例,其中男72例、女53例,平均年龄(6.4±6.3)个月。比较两组临床结果。结果全组术后随访1~11年,死亡18例,失访19例,1年、5年和10年的生存率分别为90.2%、87.0%和85.2%。其中,一期根治组死亡2例,分期手术组初期手术后死亡12例,初期手术后已行终期手术的患儿死亡4例,三尖瓣Z值(P=0.013)和右室重度发育不良(P=0.025)是影响PA/IVS患儿术后死亡的危险因素。分期手术最后完成终期手术58例,终期手术(含一期根治)占55.6%(84/151)。全组中期随访手术再干预共5例,其余患者均恢复良好,心功能分级仅2例为Ⅲ级,余均为Ⅰ~Ⅱ级。结论依据不同患儿右心室发育程度、就诊年龄以及有无冠状动脉畸形等制定个体化的外科治疗策略,可明显提高PA/IVS患儿的手术成功率,早期完成右室减压手术有利于提高双心室修补机会。     

小儿肺动脉闭锁伴室间隔缺损右室流出道重建方法

目的探讨和评价小儿肺动脉闭锁（PA）伴室间隔缺损（VSD）右室流出道重建方法的应用。方法1999年4月至2004年12月，收治81例PA伴VSD行一期或分期的双心室修补术病儿。一期根治术组32例，大部分病例采用自体心包片跨瓣环水平扩大右室流出道至肺总动脉，其中4例带心包单瓣，合并主肺动脉侧支（MAPCAs）的11例中有2例完成肺动脉血管的单元化手术。姑息手术组49例，分别采用不同的右室流出道重建方法。其中15例完成了二期根治术，大部分采用同种异体心包片跨瓣补片。结果一期根治术死亡4例（12．5％）；姑息手术死亡3例（6．1％）；二期根治术无死亡。出院时根治术组经皮氧饱和度0．95～0．98。随访3～24个月。姑息手术为0．79～0．87，McGoon指数从0．4～0．7增至1．1～1．6。结论术前判断肺动脉干、肺动脉分支发育情况、中央共汇、有无MAPCAs，对手术方法的选择极为重要。术后PRV／PLV的测定有助于右室流出道重建方法预后的评估。     

伴室间隔缺损的肺动脉闭锁的外科治疗

目的探讨伴室间隔缺损的肺动脉闭锁（VSD-PA）的外科治疗的手术时机、适应证和方法。方法1984年6月至2005年3月对32例VSD-PA进行外科手术治疗33例次,32例中男15例、女17例,年龄6个月～9岁,其中合并主动脉到肺的侧支循环动脉（APCA）的9例。3例周围肺动脉发育差、肺动脉指数（PAI）〈150mm^2／m^2,行升主动脉-肺动脉分流术;2例全部新的肺动脉指数（TNPAI）〈150mm^2／m^2或肺循环完全由分散、细小的MAPCA供应者,行一期单源化手术和室间隔缺损开放;27例（28例次,包括1例分流术后13个月）肺动脉发育达到PAI〉150mm^2／m^2或TNPAI〉200mm^2／m^2,行中度低温体外循环下一期矫治手术。结果术后早期死亡5例,其中一期矫治手术后死亡4例,分别为严重低心排血量综合征2例,灌注肺1例,多脏器功能衰竭1例;升主动脉肺动脉分流术后死亡1例,为切口感染、心内膜炎。21例随访3个月-15．5年,NYHA心功能Ⅰ和Ⅱ级19例,Ⅲ和Ⅳ级各1例。结论VSD-PA的外科治疗包括姑息或矫治手术,对于肺动脉发育较好的VSD—PA,尽可能早期进行一期矫治手术可以得到满意的效果。     

杂交技术治疗室间隔完整型肺动脉闭锁

目的 总结杂交技术经胸肺动脉瓣球囊扩张成形术治疗室间隔完整型肺动脉闭锁的即刻疗效及近、中期随访结果.方法 2005年3月至2010年3月,采用超声引导经胸肺动脉瓣球囊扩张成形术治疗室间隔完整型肺动脉膜性闭锁30例,年龄1天～48个月,平均(4.59±3.21)个月.胸骨正中切口,于右室流出道距离肺动脉瓣环下约2 cm缝荷包线,然后置入导丝.在超声引导下置入穿刺鞘管.确认穿刺针对准膜性闭锁的瓣膜后,在钢丝引导下放入球囊扩张管进行扩张,超声提示肺动脉瓣开放满意.＜3个月病婴行改良Blalock-Taussig(B-T)体肺分流术,并同期行动脉导管结扎术.＞3个月病婴行球囊扩张术后,如血氧饱和度改善明显,不常规行改良B-T分流术,并保留动脉导管开放,如血氧饱和度改善不明显,则考虑行改良B-T分流术,结扎或保留动脉导管.＞5个月病儿行球囊扩张后血氧饱和度改善不满意,且重度右心发育不良,则选择双向Glenn术.结果 30例行球囊扩张均取得成功,同期行动脉导管结扎术25例,改良B-T分流术8例,双向Glenn术2例.均未出现严重并发症.1例术后因低氧血症,术后第3天行动脉导管结扎术和改良B-T分流术;余者术后血流动力学稳定,顺利出院.术后随访1.5～62.0个月,平均(18.7±17.2)个月.血氧饱和度由术前0.73±0.08上升至0.94±0.04,心功能Ⅰ级.院外死亡5例,25例生长发育良好.结论 杂交技术经胸肺动脉瓣球囊扩张成形术是一种治疗新生儿及婴幼儿室间隔完整型肺动脉膜性闭锁的安全、有效的方法.

Abstract：

Objective In patients with pulmonary atresia and intact ventricular septum ( PAIVS) without right ventricular-dependent coronaries, catheter techniques including the use of a sniff wire, lasers, and radiofrequency have been the most widely used initial therapy. However, percutaneous perforation and balloon valvuloplasty were associated with higher rate of procedural failure and serious complications. Methods We report our experience with a hybrid approach for pulmonary atresia with intact ventricular septum, combining surgery and interventional catheterization techniques. Between March 2005 and March 2010, hybrid procedure was carried out successfully in 30 newboms and infants with favorable anatomy. The age ranged from 1 day to 48 months with a mean of (4.59 ±3.21) months. The heart was exposed through median sternotomy. A pursestring suture was placed in the right ventricular outflow tract 2 cm away from the pulmonary trunk. Then a 16-gauge intravenous catheter was punctured through the right ventrical and perforated the atretic PV with the guidance of echocardiography. A guide wire was then inserted into the sheath and used to guide the balloon across the PV. Sequential dilations were performed until a full opening of the PV with the guidance of epicardial echocardiography. In patients ＜ 3 months PDA ligation was performed followed by modified Blalock-Taussig (B-T) shunt. In patients ＞ 3 months PDA ligation was not performed. A modified B-T shunt was inserted if severe systemic oxygen desaturation occurred after PDA ligation. Bidirectional Glenn shunt was performed for severe hypoplasia. Hybrid procedure was achieved in all patients. The simultaneous procedures included 25 cases of PDA ligation. 6 newborns underwent modified B-T shunt placement (3.5 to 5 mm) after pulmonary valvuloplasty and PDA ligation, and 2 patients ＞ 1 month underwent modified B-T shunt. Another 2 patients were selected for univentricular palliative surgery because of a diminutive monopartite right ventricle and bidirectional Glenn procedure was performed. No pericardial effusion or cardiac tamponade was observed in all patients. Another case without PDA ligation underwent a modified B-T shunt because of hypoxemia three days after hybrid procedure, and the rest patients were discharged without any further surgical intervention.During the follow-up period of 1.5 to 62.0 months, 5 patients died. 25 (83.3%) survived and were all in New York Heart Association functional class 1. Peripheral oxygen saturation increased from 0.73 ± 0.08 to 0.94 ± 0.04 (P ＜ 0.05). One patient remains in a single-ventricle pathway, whereas 24 patients achieved a two-ventricle circulation. Results Conclusion Perventricular balloon pulmonary valvuloplasty using a hybrid approach is a safe and feasible procedure for patients with PAIVS.     

年长儿童及成人患者肺动脉闭锁合并室间隔缺损的外科治疗

目的探讨年长儿童和成人患者肺动脉闭锁伴室间隔缺损(PA-VSD)外科手术治疗的方法、术式和手术时机,以提高手术疗效。方法 1996年10月至2008年10月共有39例8岁以上PA-VSD患者在北京阜外心血管病医院行一期或分期根治术,其中男21例,女18例;年龄8～27岁,平均年龄13.43岁。A型14例,B型11例,C型14例。行一期根治术23例,采用同种带瓣血管或带瓣牛颈静脉行肺动脉-右心室流出道连接术,对合并粗大体动脉肺动脉侧枝血管的3例患者行肺动脉融合术(UF);分期手术16例,其中体动脉-肺动脉分流术9例,分期根治术7例。结果围术期共死亡6例,总病死率15.38%(6/39),其中一期根治术院内死亡4例(17.39%),均为C型患者,死亡原因为术后肺动脉压增高引起的低心排血量综合征;分期手术院内死亡2例(12.50%),其中1例B型再次分流患者,1例C型分期根治患者,均为再次手术中损伤主动脉,引起出血死亡。随访28例,随访率84.85%,随访时间14.0个月～9.2年,失访5例。随访期间无死亡患者,心功能I级9例,Ⅱ级13例,Ⅲ级5例,Ⅳ级1例。3例患者主动脉瓣有少量至中量反流,给予口服利尿补钾药物治疗,定期随访。结论年长儿童和成人PA-VSD患者肺血管损伤程度较重,且常合并粗大体动脉肺动脉侧枝血管,病情复杂和重症患者一期根治手术的标准应当在现有标准的基础上更加严格界定。     

室缺合并肺动脉闭锁的手术治疗

室缺合并肺动脉闭锁的手术治疗吴清玉吴洪斌室间隔缺损（ＶＳＤ）合并肺动脉闭锁比较少见，可合并其它心脏畸形，病情复杂，手术常需用右室至肺动脉外通道矫治，危险性较大。我们于１９９１年３月至１９９５年２月连续为８例病儿行根治术，近期疗效满意，报告如下：临床资...     

自体心包组织重建右室流出道-肺动脉连接治疗肺动脉闭锁并室间隔缺损的疗效分析

目的 探讨自体心包组织重建右心室流出道-肺动脉连接治疗肺动脉闭锁并室间隔缺损(pulmonary atresia with ventricular septal defect,PA/VSD)的临床效果.方法 2010年1月～2019年6月我院收治的PA/VSD病人41例;均采用自体心包组织重建右心室流出道及肺动脉,行一...     

Temporary decompression of the right ventricle to assess the right ventricle-dependent coronary circulation in pulmonary atresia with intact ventricular septum

We repaired a case of pulmonary atresia with intact ventricular septum in which the blood supply to the left anterior descending coronary artery depended on the right ventricle. At the time of a bidirectional Glenn operation, total cardiopulmonary bypass with venous drainage from the right atrium was performed in order to evaluate the safety of right ventricular decompression required for a planned Fontan operation. We confirmed the dependence of the coronary perfusion on the right ventricle by demonstrating transient depression of the ST segment in the epicardial electrocardiogram during temporary decompression of the right ventricle. To prevent ischemic myocardial damage, we then performed an extracardiac Fontan operation with a temporary venous shunt and without cardiopulmonary bypass.     

室间隔完整型肺动脉闭锁手术治疗的个体化方案

目的 总结室间隔完整型肺动脉闭锁(pulmonary atresia with intact ventricular septum,PA/IVS)的个体化手术方案.方法 回顾2004年5月至2012年5月,共收治72例PA/IVS患儿(婴),生后1天～5.2岁.平均(1.3±0.6)岁;三尖瓣(TV)中到重度反流58例;均行急诊或亚急诊手术.首次就诊非体外循环下单纯行BT分流术(A组)16例;体外循环下行肺动脉瓣切开后跨瓣补片术加或不加BT分流术(B组)33例;镶嵌治疗方法,闭式肺动脉瓣口的疏通术加或不加BT分流术(C组)22例.其中有16例已完成二期手术.结果 术后死亡10例(13.9％),其中行体外循环下行肺动脉流出道的疏通再加BT术者6例,镶嵌治疗者2例.术后随访,超声检查显示肺动脉瓣的跨瓣压差15～39 mm Hg(1 mm Hg=0.133 kPa),平均(23±5)mm Hg;三尖瓣反流明显改善,中度反流11例,轻到中度13例,其余均为轻度.术后随访2～5年,8例双心室修补,5例中度的三尖瓣反流加重到重度,右心室严重的发育不良者已行1 1/2心室修补,2例分别完成了单纯BDG和Fontan术.结论 PA/IVS的治疗方法中体肺动脉分流术(MBTS)是常用的姑息手术方法,首次就诊镶嵌治疗方法优于传统的体外循环方法纠治,随访三尖瓣的Z值和右心室发育情况决定双室修补、1 1/2心室或Fontan术.     

Anesthetic management of bidirectional cavopulmonary shunt in a patient with pulmonary atresia with intact ventricular septum associated with sinusoidal communications

Pulmonary atresia with intact ventricular septum (PAIVS) is sometimes associated with coronary artery anomalies, including right ventricle (RV)-to-coronary artery fistulas (sinusoidal communications), coronary artery stenoses, and coronary artery occlusions. In some cases, the coronary circulation depends entirely or partly on the desaturated systemic venous blood supply from the RV. Under these circumstances, decompression of the RV can result in fatal myocardial ischemia. A 6-month-old boy, diagnosed with PAIVS associated with sinusoidal communications, underwent a bidirectional cavopulmonary shunt procedure under venoarterial cardiopulmonary bypass (CPB). During CPB, to prevent RV decompression, we maintained right atrial pressure above 5 mmHg and used a pump perfusion rate of 30%–40% of the calculated value based on body surface area. Although electrocardiography showed slight ST depression and bradycardia, myocardial contractility after weaning from CPB was adequate to maintain the circulation with the administration of dobutamine and atrial pacing. In patients with PAIVS and RV-dependent coronary circulation, it is important to maintain coronary artery perfusion throughout the period of anesthesia.     

室间隔完整的肺动脉闭锁外科治疗

目的探讨适当的手术方式,评价手术矫治室间隔完整的肺动脉闭锁的临床疗效。方法1992年1月至2004年8月手术治疗17例室间隔完整的肺动脉闭锁病儿,男9例,女8例;年龄15d~12岁,平均(25.5±7.9)个月;体重3.5~28.0kg,平均(7.8±5.4)kg。其中根治手术10例,三尖瓣Z值-2.3~1.2,平均(-0.78±0.34),肺动脉发育指数149.53~297.89mm2/m2,平均(206.35±82.15)mm2/m2,其中2例病儿因严重低氧血症术后次日加行体肺分流手术;生理矫治手术6例,Z值-6.1~0.2,平均(-2.7±0.92),肺动脉发育指数39.88~218.29mm2/m2,平均(131.85±72.93)mm2/m2,包括双向格林手术2例,体肺动脉分流手术1例,右室流出道重建合并体肺动脉分流术3例;一个半心室矫治术1例,首先行双向格林手术,2年后行房间隔缺损修补术,动脉导管结扎术和右室流出道重建术。结果围术期死亡3例(16.67%),2例为先行根治术次日加行体肺分流手术者,1例为右室流出道重建合并体肺动脉分流术者。术后并发症低心排综合征3例,低氧血症3例,乳糜胸1例,右心衰3例,余者均顺利康复。结论合并室间隔完整的肺动脉闭锁是一种少见的先天性心脏病,依照右心室大小、三尖瓣环大小、肺动脉发育情况及冠状动脉解剖等采用适当的手术方式可取得满意的治疗效果。     

Surgical strategy for pulmonary atresia with intact ventricular septum: initial management and definitive surgery

Pulmonary atresia with intact ventricular septum is difficult to manage, and the surgical procedures are associated with high mortality and morbidity. The lesion is morphologically heterogeneous, with varying degrees of right ventricular and tricuspid valve hypoplasia. Aberrations of the coronary circulation are common. A single approach is impractical because the wide spectrum of right heart morphology makes a uniform surgical approach impossible. Anatomical criteria, such as the degree of tricuspid valve or right ventricular hypoplasia or whether a right ventricular infundibulum is present, have been used to assign the infants to single-ventricular or biventricular repair tracks. If the patient is considered a candidate for biventricular repair, right ventricular decompression, either alone or combined with a systemic-pulmonary artery shunt, is performed, whereas only a systemic-pulmonary artery shunt is performed in a candidate for single-ventricular repair. Several authors reported that the Z-value of the tricuspid valve diameter could be a guide to the initial procedure; however, poor interobserver reproducibility and wide normal ranges are serious limitations. Definitive operations, including biventricular repair, one-and-a-half ventricular repair, or Fontan-type operation, are performed to eliminate cyanosis. The most important problem when planning a strategy for a definitive repair is the fact that right ventricular growth and its contribution to pulmonary flow is difficult to predict before the operation. The management protocol should be determined by the precisely estimated morphology of the right ventricle and tricuspid valve, and the adequacy of the protocol should be analyzed based on surgical outcomes. This review was submitted at the invitation of the editorial committee.     

Biventricular repair for pulmonary atresia with intact ventricular septum associated with sinusoidal communications

Pulmonary atresia with intact ventricular septum (PA-IVS) is very difficult to treat due to the variety of right ventricular hypoplasia and coronary artery anomalies. Biventricular repair is viewed as possible in patients with mild right ventricular hypoplasia but not in those with severe right ventricular hypoplasia or with sinusoidal communication. We report a case of a 17-day-old boy with PA-IVS and severe right ventricular hypoplasia and large sinusoidal communications. We successfully conducted biventricular repair, ligated coronary artery and right ventricular fistulas, and implemented right ventricular outflow tract reconstruction. He now enjoys good health and attends elementary school. Sinusoidal communications are not always a limiting factor in biventricular repair for PA-IVS if the right ventricle can be decompressed without inviting ventricular dysfunction by ligating fistulas.