先天性心脏病法洛四联症并左肺动脉缺如1例

患者 ,女 ,1 5岁 ,因口唇青紫 1 4年余入院。经超声心动图检查 :法洛四联症 ,主动脉骑跨 5 0 % ,室间隔膜部缺损 2 .3cm ,右心室内径增宽、流出道变窄 ,主肺动脉发育不良、内径 1 1mm ,左肺动脉未显示 ,右肺动脉狭窄后扩张、内径 1 4mm ,左心室收缩功能指标正常、短轴缩短率 39%、射血分数 71 %、舒张末期容积 47ml;X线胸片 :胸廓对称 ,双肺纹理增多、紊乱 ,左肺门结构欠清、右肺门较大 ;右心室造影及升主动脉、降主动脉逆行造影 :主肺动脉内径 1 2mm ,右肺动脉内径 1 6mm ,主动脉内径 35mm ,未见左肺动脉 ,无动脉导管未闭。降主动脉T7段发…     

法洛四联症根治术后远期肺动脉返流问题及处理对策

法洛四联症(tetralogy of Fallot，TOF)根治术临床应用至今已40余年，在术后远期并发症中，右室功能不全、运动能力下降、心律失常性猝死是不容忽视的问题，而以上并发症都与手术中的一个重要环节——肺动脉狭窄的解除、右室-肺动脉流出道的重建有密切关系。经典的右室流出道重建方式在远期发生肺动脉返流(pulmonary regurgitation，PR)的比率很高，对患者的远期预后产生许多不利影响。本文就TOF根治术后远期PR的后果及如何最大限度地减少PR的问题作一综述。     

成人法洛四联症根治术的麻醉处理

成人法洛四联症由于心内血管畸形继发严重病变 ,长期缺氧 ,常合并有多脏器功能的损害、灌注肺、肾功能衰竭及因侧支循环丰富而术中术后易致出血、渗血等并发症。同时由于主动脉骑跨 ,高位大室间隔缺损 ,右室流出道狭窄所致右向左分流 ,肺血流减少 ,且伴有左心室发育不良 ,低氧血症和红细胞继发增多所致的血液黏稠度增加。在麻醉期间易出现缺氧发作 ,术中、术后易出现低心排血量综合征。因此麻醉的管理难度增加。我院 2 0 0 0年～ 2 0 0 4年共行 7例成人法洛四联症的手术治疗。现总结如下 :     

成人法洛四联症术后血栓外压性肺动脉狭窄介入球囊成形术一例

患者男,23岁。因＂法洛四联症矫治术后4年,间断腹胀1年余＂入院。患者4年前行＂法洛四联症矫治术（牛颈静脉跨环补片）＂,1.5年前开始间断出现腹胀、乏力,伴大量腹水、双下肢浮肿等表现。查体：心界扩大,心率100次/min,律不齐,胸骨左缘3～4肋间可闻及3/6级收缩期粗糙杂音,传导广泛,腹部稍膨隆,脐略膨出,肝肋下2指、质地软,无压痛。     

法洛四联症肺动脉形态学分析

目的:研究法洛四联症肺动脉的形态学特点。方法:利用医学影像三维重建软件,分别对10例无肺动脉畸形和10例确诊法洛四联症患儿的肺动脉进行重建,分析三维模型的形态学特征。结果:正常肺动脉的三维模型具有同质性,其俯视图呈正Y型,主肺动脉中心线走向与左肺动脉相延续;法洛四联症患儿肺动脉三维模型形态差异较大,不具有正常结构间的同质性,在模型上表现为偏移的Y型。结论:法洛四联症患儿肺动脉形态学特征存在差异,需在术前进行详细的肺动脉形态学分析。     

法洛四联症根治术后远期肺动脉返流问题及处理对策

法洛四联症(tetralogy of Fallot,TOF)根治术临床应用至今已40余年,在术后远期并发症中,右室功能不全、运动能力下降、心律失常性猝死是不容忽视的问题[1],而以上并发症都与手术中的一个重要环节--肺动脉狭窄的解除、右室-肺动脉流出道的重建有密切关系.经典的右室流出道重建方式在远期发生肺动脉返流(pulmonary regurgitation,PR)的比率很高,对患者的远期预后产生许多不利影响.本文就TOF根治术后远期PR的后果及如何最大限度地减少PR的问题作一综述.     

经右心房-肺动脉行法洛四联症一期根治术疗效分析及手术技术体会

目的 探讨经右心房肺动脉行法洛四联症一期根治术的疗效.方法 2006年6月至2010年2月,我院共采用经右心房/肺动脉（RA/PA）行法洛四联症一期根治术16例,有随访记录的14例,为右心室/肺动脉组（简称RA/PA组）;我院有术后随访记录的法洛四联症患者中,随机选取同时期采用传统手术治疗的3岁以下患者27例为传统组.结果 ①术后呼吸机辅助呼吸时间及血管活性药物应用时间,RA/PA组明显低于常规组;②术后右心功能RA/PA组较同龄人明显升高.结论 经肺动脉右心房行法洛四联症一期根治术可以缩短术后治疗时间,远期右心功能明显改善.     

法洛四联症根治术后早期并发症的临床分析与处理

目的:总结法洛四联症(tetralogy of Fallot,TOF)患儿根治术后早期处理经验,以降低TOF根治术后早期并发症的发生率及病死率。方法:回顾性分析2012年1月至12月完成的TOF根治术188例,将其分为并发症组31例,非并发症组157例。对比两组患儿年龄、体质量,术前血色素、血氧饱和度、McGoon比值,术中体外循环时间、主动脉阻断时间,术后呼吸机辅助时间、体质量监护室停留时间,正性肌力药物评分以及术后右心室流出道压力差。结果:并发症组术前McGoon比值(1.54±0.21)vs.(2.01±0.42),体外循环时间(112.54±33.32)vs.(97.03±26.1)min、主动脉阻断时间(65.38±15.41)vs.(61.87±15.38)min,呼吸机辅助时间(85.64±35.38)vs.(44.62±21.84)h、监护室停留时间5.0(2.0,7.0)vs.3.0(1.0,5.0)d,正性肌力药物评分(18.21±6.27)vs.(10.16±3.18)与非并发症组比较差异有统计学意义(P0.05)。术后右心室流出道压力差21.5(12.3,33.8)vs.24.0(17.0,32.0)mmHg(1mmHg=0.133kPa),并发症组与非并发症组比较差异无统计学意义。并发症包括低心排出量综合征(低心排)9例,渗漏综合征12例,心律失常5例,灌注肺损伤2例,感染5例。其中死亡5例(病死率2.66%)。结论:严格把握手术适应证,缩短体外循环时间,术后合理应用正性肌力药物,积极腹膜透析是预防和控制TOF根治术后低心排和渗漏综合征的有效方法。呼吸机辅助通气呼气末正压治疗及高频振荡呼吸机治疗可以控制大多数灌注肺,必要时可介入封堵侧枝。     

法洛四联症根治术后中远期常见并发症的研究进展

<正>法洛四联症(tetralogy of Fallot,TOF)是最常见的发绀型先天性心脏病,占先天性心脏病的5%～7%,TOF属于圆锥动脉干畸形,包括广义右心室流出道(right ventricular outflow tract,RVOT)狭窄、非限制性室间隔缺损(ventricular septal defect,VSD)、主动脉骑跨(骑跨范围≤50%)以及继发性右心室肥厚4种畸形^[1]。TOF的典型临床表现为紫绀、婴幼儿喂养困难、缺氧发作、大年龄患者喜蹲踞等,严重者可出现心力衰竭。超声心动图为临床首选的检查方法,可显示心脏及大血管不同方位的断层结构与毗邻关系,还可应用组织多普勒成像和心肌变形成像(如纵向应变和应变率)等技术进行功能评估,同时也是评价瓣膜反流的一线影像学检查。     

支架置入术治疗大动脉炎所致右肺动脉狭窄伴左肺动脉缺如一例

先天性一侧肺动脉缺如(unilateral absence of a pulmonary artery,UAPA)为一种罕见的心血管畸形,多与其他先天性心脏病并存。大动脉炎(takayasu’s arteritis,TA)为主动脉及其主要分支以及肺动脉或冠状动脉的慢性进行性非特异性炎症,引起血管的狭窄、堵塞或扩张。头臂动脉型、胸腹主动脉型、混合型均可合并肺动脉受累,单纯肺动脉受     

Results of the surgical repair of branch pulmonary artery stenosis in tetralogy of Fallot]

This study was based on the follow-up of 55 patients with tetralogy of Fallot and operated for branch pulmonary artery stenosis. Twenty two patients were followed up clinically (Group I): 91% of the patients were asymptomatic at the end of the first operative year. Thirty three patients (Group II) underwent control catheter studies after an average of 2.5 years after complete repair: only 70% of these were asymptomatic at 1 year; 8 were reoperated after an average of 3.6 years. The surgical result could only be evaluated by control catheter studies. The results were therefore based on the data obtained from Group II: this selection resulted in a pessimistic bias as the outcome in the two groups was significantly different. The stenoses were congenital and/or iatrogenic. They were located on the left branch (55%), the right branch (9%), the bifurcation (24%), separately on the two branches (12%). The diversity of the operative techniques and materials used reflects the difficulty of this surgery. The cumulative results were good in 33% of cases, average in 28% and poor in 39%. The results were better in isolated branch stenosis than in stenosis of the bifurcation, though not statistically significant. The results were related to age, body weight, the type, the site, the length, the association of stenoses and the material used. Significant predictive factors for a good result were: Gore-Tex (p less than 0.05) and pericardial pedicle (p less than 0.005). The average follow-up of patients with the latter type of patch was only 2 years. These results require confirmation by studies of larger numbers of patients followed up for a longer period.     

Alternative approach for angioplasty of stenosed left pulmonary artery following intracardiac repair of tetralogy of Fallot

A 5-year-old boy with stenosed left pulmonary artery following total correction of tetralogy of Fallot underwent surgical pulmonary arterioplasty through a left anterolateral thoracotomy on a normothermic perfused heart under cardiopulmonary bypass. We found this to be a convenient approach, and recommend it for correction of this lesion in the absence of gross pulmonary regurgitation and right ventricular outflow tract dilatation.     

Predictors of hemodynamically successful left pulmonary artery stent implantation in patients after repair of tetralogy of Fallot

OBJECTIVES: Left pulmonary artery stenosis is a well-known postoperative complication in patients with tetralogy of Fallot. Recently, balloon expandable intravascular stents have been widely used to relieve those lesions. However, the increase in left pulmonary blood flow is not adequate in some patients, although the vessels are suitably dilated. This study evaluated the predictors of hemodynamical improvement. METHODS: The study population consisted of nine patients with morphologically successful stent implantation for left pulmonary artery stenosis after repair of tetralogy of Fallot. Patients were divided into two groups. Four patients had hemodynamical improvement by stent implantation, with relative perfusion of the left lung of over 30% of total pulmonary perfusion. The other five patients had relative perfusion of the left lung of under 30% of total pulmonary perfusion after stent implantation. The timing of stent implantation and the morphological features of pulmonary artery were compared between the two groups. RESULTS: In hemodynamically improved patients, the stent implantation was performed earlier(6.1 +/- 3.5 vs 16.1 +/- 6.5 years old, p = 0.029), and the interval between surgical repair and stenting was shorter (3.7 +/- 2.7 vs 11.3 +/- 4.7 years, p = 0.025). There was no difference in the left pulmonary artery diameter after stenting, but the right pulmonary artery diameter was significantly smaller in hemodynamically improved patients (99.0 +/- 23.7 vs 135.0 +/- 15.1% normal, p = 0.027). CONCLUSIONS: Effective stent implantation for left pulmonary artery stenosis in patients with tetralogy of Fallot after repair must be performed before compensatory right pulmonary artery growth occurs.     

Radiofrequency perforation in the treatment of acquired left pulmonary artery atresia following repair of tetralogy of Fallot.

Acquired pulmonary artery discontinuity can complicate operative repair of certain congenital heart defects. We describe successful recanalization of acquired left pulmonary artery atresia using radiofrequency energy in a 14-month-old child who had previously undergone repair of tetralogy of Fallot with pulmonary atresia.     

Aberrant left subclavian artery in tetralogy of Fallot

The anatomy of the aberrant left subclavian artery in a right-sided aortic arch is different in patients with tetralogy of Fallot than in persons with a normal heart. In all eight patients with tetralogy of Fallot and aberrant left subclavian artery in this series, the left subclavian artery arose directly from the distal aortic arch. In contrast, normal persons with right aortic arch and aberrant left subclavian artery invariably have an aortic diverticulum from which the left subclavian artery and the left ductus arteriosus originate. The possible different embryologic events responsible for these two forms of aberrant left subclavian artery are discussed.     

Aortic stenosis after uncomplicated surgical repair of tetralogy of Fallot

Tetralogy of Fallot is only rarely associated with aortic valvar disease. We present a child who had uncomplicated repair of tetralogy of Fallot at 16 months of age, and who developed mild aortic stenosis three years later, with mild to moderate aortic incompetence being seen two years subsequent to that event. No aortic valvar disease had been noted prior to surgery. No intervention is planned at this stage.