室间隔完整型肺动脉闭锁及肺动脉瓣狭窄的手术治疗

目的探讨新生儿、婴儿室间隔完整型肺动脉闭锁及危重型肺动脉瓣狭窄的手术治疗。方法19例患儿,其中室间隔完整型肺动脉膜性闭锁10例,危重型肺动脉瓣狭窄9例。15例经胸正中切口体外循环心脏不停跳下完成手术,4例经左胸后外侧切口非体外循环下完成手术。除早期1例同期行动脉导管未闭（PDA）结扎及卵圆孔未闭缝合术外,其余18例均保留PDA、单纯切开肺动脉瓣。结果本组围术期死亡2例,院外死亡1例。其余16例术后当日肺动脉跨瓣压差为37—132mmHg,平均61mmHg;2周后为26～77mmHg,平均43mmHg,较术后早期明显降低（P〈0．05）。出院前不吸氧下测动脉血氧饱和度78％～92％,平均85％,较术前明显增高（P〈0．05）。随访PDA均闭合,右心室较术前明显增大,三尖瓣反流消失或明显减轻,房水平分流消失。结论对室间隔完整型肺动脉闭锁及危重型肺动脉瓣狭窄实施单纯肺动脉瓣切开术治疗安全有效。     

单纯肺动脉瓣疏通术初期治疗危重婴幼儿室间隔完整型肺动脉闭锁

目的:探讨低龄危重婴幼儿室间隔完整型肺动脉闭锁,及危重肺动脉瓣狭窄的初期外科治疗方法。方法:2009年6月至2013年6月,我院共对10例室间隔完整型肺动脉闭锁及2例危重肺动脉瓣狭窄婴幼儿患者,施行经胸正中切口非体外循环下,单纯肺动脉瓣疏通术(改良Brock术)治疗,术中保留动脉导管未闭,三尖瓣反流未做处理。患儿年龄2~9个月,体质量4.3~10kg,平均6.4kg。所有患者均合并动脉导管未闭及房间隔缺损或者卵圆孔未闭,术前患者末梢血氧饱和度62%~73%,平均69%。结果:本组围术期死亡1例(8.3%),死亡原因为术后持续低氧血症,其余11例术后均缺氧状态改善,经皮血氧饱和度上升至84%~93%,平均89%。术后测得肺动脉瓣跨瓣压差为11~28mmHg(1mmHg=0.133kPa),平均15.6mmHg,术后随访11例,PDA均闭合,三尖瓣反流由术前大量转为少量或者消失。结论:作为危重婴幼儿的初期治疗,对于发绀严重,生长发育差,不能耐受体外循环的危重室间隔完整型肺动脉闭锁及危重肺动脉瓣狭窄患儿,Brock术可获得满意的治疗效果。     

室间隔完整型肺动脉闭锁的手术治疗(附8例报告)

目的总结婴幼儿室间隔完整型肺动脉闭锁的手术治疗经验。方法回顾性分析8例室间隔完整型肺动脉闭锁患儿的手术资料。结果根据Z值选择合适的手术方案,其中非体外循环下肺动脉瓣闭式扩张术6例;体外循环下行右心室肺动脉干重建术+房间隔修补术1例;体肺动脉分流术+部分双心室修补术1例。围手术期死亡1。其余7例术后当日及两周后肺动脉跨瓣压差明显降低),随访1个月～7 a,右心室功能较术前均有明显改善,经皮血氧饱和度均大于0.95。结论根据室间隔完整型肺动脉闭锁患儿Z值选择个体化的手术方案积极手术效果满意。     

小儿复合先天性心脏病的介入治疗

目的:探讨小儿复合先天性心脏病(先心病)经导管介入治疗的策略及评价其疗效和安全性.方法:55例复合先心病患儿,先心病类型包括:肺动脉瓣狭窄(PS)合并房间隔缺损(ASD);PS合并动脉导管未闭(PDA);PS合并室间隔缺损(VSD);ASD合并PDA;ASD合并VSD;PDA合并VSD;室间隔完整的肺动脉闭锁(PA/IVS)合并PDA和卵圆孔未闭(PFO);主动脉瓣狭窄合并PDA;PDA合并肺隔离症.分别行射频打孔,经皮球囊肺动脉瓣成形术(PBPV),经皮球囊主动脉瓣成形术(PBAV),VSD封堵术,PDA封堵术,ASD封堵术,侧支血管堵塞术.结果:55例患儿经导管介入治疗均获得成功,术中未发生严重并发症,55例行介入堵闭均未见残余分流,堵闭器位置良好;30例行PBPV术,跨肺动脉瓣压差由术前平均(63.4±36.3)mmHg(1 mmHg=0.133 kPa),下降到术后平均(18.1±13.0)mmHg(P<0.01);1例行PBAV术,跨主动脉瓣压差由术前90 mmHg下降到术后50 mmHg;3例PA/IVS行射频打孔和PBPV术,术后肺动脉瓣开放满意,血氧饱和度维持良好.结论:复合先心病经导管介入治疗疗效确切、安全有效,但手术操作难度大,技术要求高,应该在较大心血管医院开展,并由有较丰富导管操作经验的医师施行.     

室间隔完整型肺动脉闭锁及危重型肺动脉瓣狭窄的介入治疗进展

室间隔完整型肺动脉闭锁与危重型肺动脉瓣狭窄是不常见的发绀型先天性心脏病,随着诊断技术的不断提高与介入治疗的逐步开展,这两种疾病的预后得到了明显的改善。本文旨在回顾室间隔完整型肺动脉闭锁、危重型肺动脉瓣狭窄的介入治疗进展,为这两种疾病的治疗策略提供一些参考。     

经皮球囊肺动脉瓣成形术治疗婴儿重度肺动脉瓣狭窄及室间隔完整的肺动脉瓣闭锁

目的:总结经皮球囊肺动脉瓣成形术(percutaneous balloon pulmonary valvuloplasty,PBPV)治疗婴儿重度肺动脉瓣狭窄(pulmonary stenosis,PS)及室间隔完整的肺动脉瓣闭锁(pulmonary atresiawith intact ventricular septum,PA/IVS)的经验,评价其疗效及安全性。方法:自2007年1月至2012年10月,采用PBPV治疗婴儿重度PS及PA/IVS患者共33例,男性24例,女性9例,手术年龄3～12(8.9±2.9)个月,体质量5～11.5(9.0±1.6)kg。术前完善心电图、X线片、超声心动图、右心导管检查及右心室造影,采用单球囊扩张完成PBPV。PA/IVS患儿需先行肺动脉瓣射频打孔术。结果:球囊扩张手术技术成功率为96.97%(32/33)。右心室收缩压(right ventricular systolic piessure,RVSP)由术前的95～205(130.8±28.2)mmHg(1 mmHg=0.133kPa)下降至28～135(73.2±27.4)mmHg(t=12.067,P<0.001);肺动脉瓣跨瓣压差由术前的81～180(110.3±26.3)mmHg下降至10～112(47.7±23.8)mmHg(t=12.958,P<0.001)。1例术中出现心脏压塞,转外科急诊手术,术后恢复良好。出院时10例患者复查超声心动图仍显示平均跨肺动脉瓣压差>50 mmHg;随访中值时间26个月,3例残余重度肺动脉瓣狭窄,2例接受二次PBPV后压差降至轻度。本组术后发生轻度以上肺动脉瓣关闭不全(pulmonary insufficiency,PI)22例。结论:随着介入技术的不断提高,经皮肺动脉瓣球囊扩张术,已成为救治婴儿危重先天性肺动脉瓣狭窄及肺动脉瓣闭锁的安全有效的重要方法。     

经导管同期治疗复合型先天性心脏病的疗效和安全性

目的:探讨经导管介入同期治疗复合型先天性心脏病的可行性、方法及疗效.方法:13例患者,男5例,女8例.其中房间隔缺损(ASD)合并肺动脉瓣狭窄6例,ASD合并动脉导管未闭(PDA)4例,ASD合并膜部室间隔缺损2例,PDA合并肺动脉瓣狭窄1例.ASD合并其他畸形的患者,均先治疗其他畸形,最后行ASD封堵.PDA合并肺动脉瓣狭窄者,先行肺动脉瓣狭窄球囊扩张术,最后封堵PDA.结果:13例患者均经导管一次治疗成功.ASD合并肺动脉瓣狭窄6例,跨肺动脉瓣压差由术前平均(138.3±41.4)mmHg下降到术后平均(12.0±5.6)mmHg,有显著性差异(P＜0.01).所用ASD封堵器直径为8～24 mm.ASD合并PDA4例,PDA最窄径2～5 mm,封堵器直径为6～8 mm;ASD封堵器直径为8～28 mm.ASD合并膜部室间隔缺损2例,室间隔缺损直径分别为3.5 mm和5.0 mm,用直径6 mm和8 mm的双盘状室间隔缺损封堵器封堵成功,所用ASD封堵器的直径为12 mm和18 mm.1例PDA合并轻度肺动脉瓣狭窄,用聚乙烯球囊扩张、PDA用4 mm封堵器治疗成功.结论:经导管介入同期治疗复合型先天性心脏病具有技术上的可行性、安全性和良好的治疗效果.     

超声心动图对室间隔完整型肺动脉闭锁的诊断价值

目的分析超声心动图对室间隔完整型肺动脉闭锁(PAIVS)的诊断价值。方法选取2013年9月—2018年5月广西北海人民医院、贵州安顺妇幼保健医院及首都儿科研究所附属儿童医院收治的PAIVS患儿25例,均进行超声心动图检查,分析PAIVS检出率及其表现。结果 (1)超声心动图检出PAIVS患儿23例,检出率为92.0%;漏诊2例,漏诊率为8.0%。25例患儿室间隔均完整,其中经心外科手术确诊19例,经心血管造影确诊4例,经心脏CT确诊3例。(2)肺动脉瓣膜性闭锁20例,肺动脉瓣消失及主肺动脉近端闭锁5例,其中"右心室依赖型"冠状动脉循环3例。(3)右心室发育不良者12例,占48.0%。(4)合并房间隔缺损或卵圆孔未闭25例(占100.0%),右房室瓣反流23例(占92.0%),动脉导管未闭18例(占72.0%),右房室瓣发育不良10例(占40.0%),体肺侧支7例(占28.0%),右房室瓣闭锁2例(占8.0%)。结论超声心动图对PAIVS具有较高的诊断价值;PAIVS主要类型为肺动脉瓣膜性闭锁,患儿常伴有右心室发育不良、房间隔缺损或卵圆孔未闭、右房室瓣返流、动脉导管未闭等。     

57例先天性心脏病复合畸形介入治疗临床评价

目的:研究先天性心脏病复合畸形经导管介入治疗策略并评价其疗效。方法:先天性心脏病复合畸形57例,复合类型为:膜部室间隔缺损(VSD)并房间隔缺损(ASD)21例,VSD并动脉导管未闭(PDA)4例,ASD并PDA8例,ASD并肺动脉瓣狭窄(PS)22例,VSD并PS2例。复合畸形同期介入治疗顺序依次为:PS球囊扩张术,VSD封堵术,PDA封堵术,最后行ASD封堵术。结果:57例患者中,除1例因PDA过小,导丝无法通过未闭导管,在VSD成功封堵后放弃PDA封堵。56例患者同期经导管介入治疗均获成功,术中未发生任何重要并发症。术后造影及超声检查VSD、ASD及PDA均无残余分流,封堵器固定好。并发PS的24例患者,跨肺动脉瓣压差由术前(96.0±30.0)mmHg(1mmHg=0.133kPa)下降到术后平均(15.6±3.3)mmHg(P<0.01)。术后3d、1个月、6个月及1年分别行经胸超声心动图、心电图、X线检查,全部患者无残余分流,封堵器位置固定良好,跨瓣压差均在正常范围内,无任何并发症发生。结论:先天性心脏病复合畸形经导管介入治疗安全有效,值得推广。     

非体外循环与体外循环下肺动脉瓣切开术治疗室间隔完整型肺动脉闭锁的疗效对比研究

目的 通过评价非体外循环和体外循环下肺动脉瓣切开术作为初期手术治疗室间隔完整型肺动脉闭锁(PA/IVS)的临床状况,比较两种方法的手术疗效和早中期预后效果.方法 回顾2015年1月至2020年12月,共45例PA/IVS患儿在我院接受非体外循环或体外循环下肺动脉瓣切开术,分为非体外循环组(28例)和体外循环组(17例)...     

Balloon pulmonary valvuloplasty after initial pulmonary valvotomy for pulmonary atresia with intact ventricular septum]

Seven patients with pulmonary atresia and intact ventricular septum have undergone closed pulmonary valvotomy on the early neonatal period (2-6 days) with satisfactory results. Follow-up catheterization showed significant residual pulmonary valve stenosis requiring a second operation. Percutaneous balloon dilatation of the pulmonary valve was successful in 6 cases with the mean right ventricular systolic pressure reduced from 6.8 to 4.9 kPa (50.8 to 37 mmHg) and the mean transvalvular gradient dropped from 5.7 to 2.1 kPa (4.28 to 15.8 mmHg). One case failed and required surgical treatment. The results showed that balloon valvuloplasty could be applied instead of surgery for treatment of selected cases of pulmonary atresia with intact ventricular septum after an initial pulmonary valvotomy.     

Longevity of Neonatal Ductal Stenting for Congenital Heart Diseases with Duct‐dependent Pulmonary Circulation

Introduction. Ductal stent (DS) in duct‐dependent pulmonary circulation is less morbid than neonatal Blalock–Taussig shunt. However, there is concern if DS provides an adequately long palliation before definitive repair. Methods. This is a retrospective review of clinical follow‐up of all consecutive infants after successful DS performed by a single operator. They were divided into three anatomic groups. Group A neonates had balloon valvotomy for critical pulmonary stenosis or pulmonary atresia with intact ventricular septum, who needed DS patency until the right ventricle was adequate to provide antegrade pulmonary flows. Group B patients with tetralogy of Fallot and pulmonary atresia suited for later biventricular repair needed ductal patency until conduit surgery was completed. Group C patients with functionally univentricular hearts needed DS patency until bidirectional Glenn shunt completion. Results. Among 22 infants, four Group A patients followed for 26–54 months after balloon pulmonary valvotomy had adequate oxygen saturation and needed only short‐term DS patency. In six out of nine Group B patients, corrective biventricular repair using conduits was performed after 5–14 months at a body weight of 5–7.5 kg. Bidirectional Glenn shunt and confluence repair were performed in seven of nine Group C patients weighing 6–8.5 kg after 8–15 months. The hilar pulmonary artery growth in B and C groups was adequate for surgical repair. No patient needed stent redilatations or additional shunts on follow‐up for hypoxia. Four patients had sudden death. Conclusions. The short‐term patency of DS was adequate after balloon valvotomy for critical pulmonary stenosis or pulmonary atresia with intact ventricular septum. Duration of palliation by DS was also sufficient in univentricular hearts to allow adequate somatic growth before Glenn surgery. In patients with biventricular anatomy treated by DS, conduit repair had to be performed at a relatively early age. Interstage mortality was 18%.     

Determinants of successful balloon valvotomy in infants with critical pulmonary stenosis or membranous pulmonary atresia with intact ventricular septum

Objectives. This study reviewed our experience with percutaneous balloon valvotomy in infants with critical pulmonary stenosis or membranous pulmonary atresia with intact ventricular septum and defined the anatomic and hemodynamic characteristics of infants in whom this procedure is successful and provides definitive therapy.Background. Unlike children with valvular pulmonary stenosis, the follow-up of infants with critical pulmonary stenosis undergoing percutaneous balloon valvotomy is limited.Methods. Between December 1987 and August 1992, percutaneous balloon valvotomy was attempted in 12 infants with critical pulmonary stenosis (n = 10) or pulmonary atresia with intact ventricular septum (n = 2). Two outcome groups were identified: Group A patients are acyanotic, have mild residual pulmonary stenosis and have not required operation; Group B patients have required operation.Results. Of the 12 infants, 11 had a successful balloon valvotomy procedure. Group A patients (n = 7) have a residual gradient of 22 ± 18.7 mm Hg (mean ± SD) at follow-up of 3.2 years (range 1.2 to 5.0). In Group B (n = 5), operation was required for inability to cross the pulmonary valve (n = 1) or persistent severe hypoxemia for ⪰2 weeks after valvotomy (n = 4). Significant differences (p ≤0.01) between the two groups (Group A vs. Group B) were identified in pulmonary valve annulus (Z value) 8.1 mm (−1.1) versus 5.5 mm (−3.4); tricuspid valve annulus (Z value) 14.0 mm (0.8) versus 8.8 mm (−1.8); right ventricular volume 65 versus 29 ml/m²; and Lewis index 10.9 versus 8.9.Conclusions. Percutaneous balloon valvotomy is effective and likely to provide definitive therapy in infants with critical pulmonary stenosis or membranous pulmonary atresia with intact ventricular septum who have a tricuspid valve annulus >11 mm, pulmonary valve annulus ⪰7 mm and right ventricular volume >30 ml/m².     

室间隔完整型肺动脉闭锁的彩色多普勒超声诊断

目的 探讨室间隔完整型肺动脉闭锁（PAIVS）的超声影像特点.方法 回顾分析7例PAIVS的超声心动图表现,并与外科手术结果进行比较.结果 PAIVS的超声心动图特征明显：（1）二维超声心动图胸骨旁左心室长轴切面显示室间隔回声完整.（2）心尖四腔切面显示2个心房正位,室间隔回声完整.（3）胸骨旁大动脉短轴切面显示2条大动脉位置关系正常,肺动脉瓣无开放运动,肺动脉瓣无血流信号通过.手术证实7例PAIVS中肺血由未闭的动脉导管供应者6例;主肺动脉及分支发育不良,仅有体肺动脉侧支供应肺血者1例.心房水平右向左分流者7例：其中5例为卵圆孔未闭,2例为房间隔缺损.合并畸形包括三尖瓣闭锁,三尖瓣发育不良伴狭窄、房间隔膨凸瘤等.结论 PAIVS患儿肺血来源多样化,肺动脉发育程度不一,超声心动图特征明显,对PAIVS有特异性诊断价值.     

Right ventricular growth in a case of pulmonic stenosis with intact ventricular septum and hypoplastic right ventricle.

Adequate growth of the hypoplastic right ventricle in a patient with severe pulmonary stenosis with an intact ventricular septum was documented after pulmonary valvotomy in infancy. It is postulated that the growth of the ventricular chamber is largely the result of pulmonary regurgitation resulting from successful pulmonary valvotomy. Based on this and the observations of others on the growth of the hypoplastic right ventricle in pulmonary atresia (with intact septum) cases, an organized approach to eventual total surgical correction is recommended.     

经皮球囊肺动脉瓣成形术204例临床分析

目的:探讨经皮球囊肺动脉瓣成形术(PBPV),治疗肺动脉瓣狭窄(PS)及室间隔完整的肺动脉瓣闭锁(PA/IVS)的安全性及有效性。方法:我科自1987年4月至2011年5月收治的PS 202例,PA/IVS 2例,男性116例,女性88例,年龄3个月～40岁,平均4.25岁,体质量5～60 kg,平均(17.32±8.85)kg,发绀34例。完善心电图、心脏X线像、超声心动图检查,行右心导管检查及右心室造影并完成PBPV。结果:PBPV 204例,成功200例,成功率98%。PBPV术后,导管测肺动脉瓣跨瓣压差〔(30.84±15.05)mmHg(1 mmHg=0.133 kPa)〕较术前(75.75±30.04)mmHg明显下降,P=0.001。4例失败病例中,1例因右心室流出道狭窄导丝难以到达肺动脉,取消PBPV。第2例导丝送入肺动脉出现循环不稳定取消PBPV。第3例PA/IVS,射频打孔成功后球囊扩张时出现心包填塞,转外科手术。第4例术中球囊扩张时右心室流出道撕裂致心包填塞,抢救无效死亡。其中2例出现严重并发症,1例三尖瓣腱索断裂;1例缺血、缺氧性脑病。结论:PBPV治疗PS及PA/IVS安全有效。严格掌握适应证,规范操作可以减少并发症。     

Severe tricuspid stenosis presenting as tricuspid atresia. Echocargraphic diagnosis and surgical management.

Two cases of pulmonary atresia with intact ventricular septum and severe tricuspid stenosis are described in which the initial angiographic diagnosis was tricuspid atresia and pulmonary atresia. Two dimensional echocardiography showed the features of an imperforate tricuspid valve because in each case contrast echocardiography failed to show anterograde flow from the right atrium to right ventricle. Successful radical surgical repair was achieved in one patient with performing a tricuspid valvotomy and inserting an external valved conduit between the hypoplastic right ventricle and the main pulmonary artery. In the second case, an infant died four weeks after tricuspid valvotomy and right ventricular outflow tract reconstruction.     

Laser valvotomy with balloon valvoplasty for pulmonary atresia with intact ventricular septum: five years' experience.

OBJECTIVE: To assess immediate and medium term results of transcatheter laser valvotomy with balloon valvoplasty in selected infants with pulmonary atresia and intact ventricular septum. DESIGN: Prospective study. SETTING: Tertiary cardiac unit. PATIENTS: All infants with pulmonary atresia and intact septum with no more than minor tricuspid valve hypoplasia referred between November 1990 and June 1995. Laser valvotomy was attempted in nine infants of median age 4-5 days and median weight 3.6 kg. INTERVENTION: The pulmonary valve was perforated using a 0.018 inch fibreoptic guidewire attached to a NdYag laser and introduced through a catheter positioned beneath the valve. After perforation the valve was dilated with progressively larger balloons. MAIN OUTCOME MEASURES: Successful laser valvotomy and balloon dilatation, complications, pulse oximetry, right ventricular outflow velocities, and need for surgical treatment. RESULTS: Valvotomy was successful in all but one case, the failure being due to laser breakdown. After perforation the valve was dilated to 6-8 mm diameter. Prostaglandin E was withdrawn immediately in six of the eight duct dependent infants, and 28 and 49 days later in two. No patient required an aortopulmonary shunt. Two patients had repeat valvoplasty at 20 days and three months of age, respectively; one required infundibular resection and closure of the atrial septum at age four and one is awaiting similar treatment. CONCLUSIONS: Laser valvotomy with balloon valvoplasty is safe and effective treatment for selected patients with pulmonary atresia and intact ventricular septum and should be considered as first line treatment in place of surgical valvotomy.