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。     

室间隔完整型肺动脉闭锁非体外循环下初期外科治疗的临床研究

目的:通过采用非体外循环直视下肺动脉瓣切开或镶嵌治疗的方法作为初期手术方案,评估治疗室间隔完整型肺动脉闭锁(PA/IVS)的手术疗效。方法:回顾性分析自2010年1月至2016年6月,在我中心完成初期手术的47例PA/IVS患者的临床资料,其中,行肺动脉瓣闭式切开术的25例患儿为肺动脉瓣(PV)切开组,行经右心室肺动脉瓣穿刺球囊扩张术的22例患者为镶嵌治疗组。对各组患儿术前彩色超声报告,手术方案,术后并发症,以及术后随访情况等予以分析统计。结果:PV切开组和镶嵌治疗组患儿术后早期病死率分别为12.0%及4.5%,再次手术干预率分别为10.5%及18.2%。术后随访中PV切开组有12例患儿完成终期手术,包括10例双心室修补,2例1.5心室修补;镶嵌治疗组有11例患儿完成中期手术,其中9例完成双心室修补,2例完成1.5心室修补。结论:非体外下初期外科手术治疗PAIVS,具有操作简便、手术时间短、心肌损伤小、手术成功率高、术后恢复快以及再干预率低等优势。采用非体外直视下肺动脉瓣切开术或外科镶嵌治疗的方法,均可获得良好的治疗效果,为患儿分期手术创造良好条件。     

婴幼儿期心室间隔完整型肺动脉闭锁的外科治疗

目的：报告４例心室间隔完整型肺动脉闭锁（ＰＡ＋ＩＶＳ）婴幼儿的一期手术根治结果，讨论此病的外科治疗。方法：４例患儿均为男婴，肺动脉闭锁部位均发生在肺动脉瓣水平，右心室３个部分都存在，分别于出生后３个月、１０个月、１１个月和２３个月在体外循环下行一期根治。结果：４例患儿均顺利度过了围术期，无并发症及死亡，根治效果满意。结论：心室间隔完整的肺动脉闭锁，如闭锁发生在肺动脉瓣水平且右心室３个部分均存在，可在新生儿或婴幼儿期行一期根治。     

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)...     

新生儿及婴儿危重型肺动脉狭窄及肺动脉膜性闭锁的外科治疗

目的:总结我科1997年4月至2007年8月对新生儿及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经验。方法:手术治疗共23例,年龄6d~11个月。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13例。19例经胸正中切口体外循环下心脏不停跳完成手术,4例应用左胸后外侧切口非体外循环方法。除早期1例同期行动脉导管未闭(PDA)结扎及卵圆孔未闭(PFO)缝合外,其余22例均采用保留PDA、单纯切开肺动脉瓣的方法。结果:围手术期死亡2例,分别死于低氧血症及急性肾功能衰竭。术后当日超声心动图测肺动脉跨瓣压差为37~132mmHg(1mmHg=0.133kPa),平均61mmHg。2周后复查示肺动脉跨瓣压差为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4个月至10年,平均5.8年。PDA均闭合,肺动脉血流通畅,三尖瓣返流消失或明显减轻。结论:保留动脉导管、单纯肺动脉瓣切开术对于治疗新生儿及婴儿室间隔完整型肺动脉膜性闭锁及危重型肺动脉瓣狭窄是一种安全有效的方法。     

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。     

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.     

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。     

动脉导管内支架置入术治疗新生儿室间隔完整型肺动脉闭锁的疗效

目的 评估动脉导管内支架置入术治疗新生儿室间隔完整型肺动脉闭锁的可行性和疗效.方法 选择2007年12月至2010年9月行动脉导管内支架置入术的室间隔完整型肺动脉闭锁新生儿11例.患儿年龄3～13(8.20±2.90)d,体重3.00～3.88(3.41±0.29)kg.根据血管造影结果选择冠状动脉支架和球囊,支架准确定位后充盈球囊扩张支架.术后观察患儿的血氧饱和度,检查超声心动图和正侧位胸片,记录支架的内径、位置,并且在支架术后1、3、6和12个月进行随访.结果 11例患儿均顺利置入支架.术前末梢血氧饱和度为(63.27±8.47)%,术前应用前列地尔后末梢血氧饱和度为(82.73±5.59)%,支架置入术后末梢血氧饱和度为(86.18±3.19)%,应用前列地尔后和支架置入术后末梢血氧饱和度均高于术前(P均<0.01),支架置入术后末梢血氧饱和度高于前列地尔应用后(P<0.05).术中测量动脉导管最窄内径为(1.69±0.37)mm,长度为(16.72±2.37)mm,置入支架内径均为4 mm,长度(20.18±3.40)mm.1例患儿在术后发生支架移位和血氧饱和度下降,并行B-T分流术;1例患儿随访期间在院外死亡,原因不明;1例患儿因术后4个月末梢血氧饱和度下降而行支架球囊扩张术;2例患儿分别在支架置入术后5和7个月行外科手术.结论 动脉导管内支架置入术治疗新生儿室间隔完整型肺动脉闭锁可行,且近期疗效良好,可作为室间隔完整型肺动脉闭锁一期手术的首选治疗方法.

Abstract：

Objective To evaluate the feasibility and efficacy of arterial duct stenting in neonates with pulmonary atresia and intact ventricular septum.Methods Eleven neonatal pulmonary atresia with intact ventricular septum patients received arterial duct stenting in our hospital from December 2007 to September 2010 were involved in this study. The average age was (8.20±2.90) days (ranged from 3 to 13 days). The average weight was (3.41±0.29) kg (ranged from 3.00 to 3.88 kg). The stents were selected according to digital subtracted angiography measurements. After checking for correct position by angiography, the balloon was inflated to expand the stent to desired diameter. Oxygen saturation was monitored, echocardiography was measured and stent diameter and location were observed by chest X-ray.Patients were followed up at 1, 3, 6 and 12 months post procedure. Results Stents were successfully implanted in all 11 patients. The preoperative peripheral oxygen saturation was (63.27±8.47)%, while increased to (82.73±5.59)% after alprostadil application and to (86.18±3.19)% after operation (all P<0.01).After the operation, the peripheral oxygen saturation was higher than alprostadil application (P<0.05).The intraoperative narrowest diameter of patent ductus arteriosus was (1.69±0.37)mm, the length was (16.72±2.37)mm. The internal diameter of implant stents was 4 mm, the length was (20.18±3.40)mm. After the operation, surgical B-T shunt operation was performed in one patient due to stent shift and pulse oxygen saturation decrease.One patient died post operation with unknown reason, another patient received stent balloon dilatation due to pulse oxygen saturation decrease at 4 months after the surgery. Pulmonary atresia with intact ventricular septum surgeries were performed in 2 patients at 5 and 7 months after stent implantation. Conclusion The neonatal pulmonary atresia with intact ventricular septum arterial stent implantation was a feasible and effective procedure and this method could be used as preferred treatment in pulmonary atresia and intact ventricular septum for neonates.     

经导管射频打孔肺动脉瓣球囊扩张治疗室间隔完整的肺动脉闭锁1例

室间隔完整的肺动脉闭锁（PA／IVS）患儿生后严重缺氧，病死率高。21世纪90年代开始应用激光或射频打孔球囊扩张介入治疗PA／IVS，我院于2009年3月18日成功地经导管射频打孔、肺动脉瓣球囊扩张介入治疗PA／IVS1例，报道如下。     

Transcatheter coil occlusion of coronary artery to right ventricular fistulae in pulmonary atresia with intact ventricular septum

We describe a young adult with an underlying diagnosis of pulmonary atresia with intact ventricular septum and Fontan physiology who presented with extreme dyspnoea on exertion. Coronary artery to right ventricular fistulae and reversible myocardial ischaemia secondary to a right ventricular steal phenomenon were present. The right coronary artery to right ventricular fistula was completely occluded with coils, by a transcoronary approach, with amelioration of symptoms. However, one coil embolised and lodged uneventfully in the left atrium during the procedure. This case demonstrates that a transcatheter approach may be successfully employed not only in patients with isolated coronary artery fistulae, but also in patients with coexistent complex congenital heart disease.     

Persistent pulmonary hypertension of the newborn associated with pulmonary atresia and intact interventricular septum

Neonates with pulmonary atresia and intact interventricular septum (PAIVS) do not have pulmonary vascular disease secondary to their heart abnormality. Persistent pulmonary hypertension of the newborn has not been described in association with this condition. The case is reported of a female neonate born with PAIVS, who preoperatively had no clinical evidence or any risk factors for persistent pulmonary hypertension of the newborn, but whose postoperative course was highly suggestive of persistent pulmonary hypertension; necropsy confirmed the features of pulmonary vascular disease.


Keywords: persistent pulmonary hypertension; pulmonary atresia and intact ventricular septum; pulmonary vascular disease; surgery; congenital heart defects     

Transcatheter perforation of atretic pulmonary valve by the stiff end of a coronary wire in neonates with pulmonary atresia with intact ventricular septum: A solution in developing countries

Objectives

To evaluate the safety of using the stiff end of a coronary wire to perforate an atretic pulmonary valve (PV) in patients with pulmonary atresia with intact ventricular septum (PAIVS).

Coronary artery fistula embolization in an infant with pulmonary atresia intact ventricular septum: a case report.

This report presents a case of pulmonary atresia/intact ventricular septum with right ventricular-dependent coronary circulation. At 7 months of age, the infant underwent coil embolization of the connection between the right ventricle and the coronary circulation. The child is currently well following surgical decompression of the right ventricle.     

Achieving biventricular circulation in patients with moderate hypoplastic right ventricle in pulmonary atresia intact ventricular septum after transcatheter pulmonary valve perforation

Objective: Transcatheter valve perforation for pulmonary atresia intact ventricular septum is the standard of care for patients with mild right ventricular hypoplasia. However, its role in moderate right ventricular hypoplasia has been less well defined. We sought to report the long‐term outcome of patients with moderate hypoplastic right ventricle who had undergone the procedure.
Design, Settings, and Patients: We performed a retrospective analysis on patients who had undergone transcatheter pulmonary valve perforation from January 1996 to January 2015 at our institution. The procedures would be carried out irrespective of the right ventricular size, as long as there were no absolute contraindications. Intervention and Outcome Measures: Demographic and procedural data were cor‐ related with outcome measures. Outcomes analyzed included procedural success, reintervention rates, final circulation type, and functional class. Multivariate analysis and receiver operator curve were used to identify for parameters in predicting biven‐ tricular circulation.
Results: The procedural success rate was 92% (33 out of 36) in this group with mod‐ erate right ventricular hypoplasia (tricuspid valve z score −4.2 ± 3.0, 69.4% of pa‐ tients with z score <−2.5). Early reintervention rate was 39%, mostly being insertion of modified Blalock–Taussig shunt. Overall reintervention‐free survival was 53%, 30%, and 19% at 1, 6, and 12 months postintervention. Despite no significant catch‐ up right ventricular growth, majority of survivors (84%) enjoyed a biventricular circu‐ lation with good functional status. A tricuspid to mitral valve ratio >0.79 was a good predictor of biventricular outcome. (specificity of 100%, positive predictive value 100%).
Conclusion: Encouraging long‐term results with biventricular circulation and func‐ tional status were demonstrated with transcatheter pulmonary valve perforation in patients even with moderate hypoplastic right ventricle, which is comparable to that with mild right ventricular hypertrophy. The baseline tricuspid to mitral valve ratio was identified as a potentially useful tool in predicting biventricular circulation.     

Does hypoplasia of one pulmonary artery preclude a definitive repair in pulmonary atresia,intact ventricular septum,and hypoplastic right ventricle?

Summary Twins with pulmonary atresia, intact ventricular septum, and hypoplastic right ventricle associated with underdeveloped and discontinuous left pulmonary artery are described. Operations to connect the left pulmonary artery to the main pulmonary trunk, with subsequent biventricular repair assisted by bidirectional cavopulmonary anastomosis and atrial fenestration were performed. Follow-up catheterization proved spontaneous closure of the fenestration in one patient. Both patients have been in a satisfactory condition for 3 years since operation.