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…     

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.     

法洛四联症根治术中左肺动脉狭窄的处理

目的:总结合并左肺动脉狭窄的法洛四联症(TOF)不同手术处理方法及随访结果。方法:回顾性分析2011年1月至2017年12月间,合并左肺动脉狭窄的TOF根治手术49例结果。患儿男性22例,女性27例,年龄7～71个月,平均19. 5个月,体质量6. 5～20. 8 kg,平均(10. 9±4. 8) kg。对于左肺动脉狭窄采用三种不同的处理方法:①常规心包补片加宽(31例)、②无线缝合心包补片加宽(10例)、③不加宽(8例)。观察术后早期及中远期随访情况。结果:死亡2例(病死率4. 1%)。超声心动图随访5～75个月,平均(27. 2±13. 6)个月,患儿肺动脉均有不同程度生长,左肺动脉压差较术前减轻。常规心包加宽组、无线缝合组及不加宽组左肺动脉处仍各有3例(9. 7%)、2例(20%)及4例(50%)存在明显压差。结论:左肺动脉狭窄影响TOF根治术结果。心包补片加宽修补左肺动脉狭窄结果满意,无线缝合适用于重度肺动脉狭窄患儿。     

法洛四联症合并右肺动脉起源异常并延迟关胸一例

法洛四联症合并右肺动脉起源异常并延迟关胸     

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。     

肺动脉发育不良型法洛四联症外科分期治疗的临床体会

目的:总结肺动脉发育不良重症法洛四联症的外科治疗经验。方法:自2002年12月至2013年12月,共矫治例肺动脉发育不良型法洛四联症33例,其中男性22例,女性11例,根治手术时年龄12~282个月,平均(40±47)个月,体质量7~34kg,平均(14±5)kg。在根治手术前进行了一次或一次以上的姑息手术。合并心血管畸形包括:房间隔缺损8例,动脉导管未闭4例,左肺动脉缺如2例,左肺动脉起自主动脉1例,永存左上腔静脉3例,合并粗大体肺侧枝血管12例。第一次姑息手术至根治手术时间间隔7~40个月,平均19个月。根治手术前行一次姑息手术者20例,2次者8例,3次者5例。姑息手术术式包括改良Blalock-Taussig分流术、改良Waterston分流术、右心室流出道重建术、肺动脉瓣球囊扩张术、肺动脉环缩以及侧枝血管结扎融合或介入封堵。结果:全组死亡1例,为根治手术后严重感染死亡,1例在首次姑息手术时因人工血管堵塞在术后第一天再次行体肺分流术,患儿根治手术前Nakata指数和Mc Goon比值分别为[(230±90)和(1.91±0.45)mm2/M2,均较姑息手术前(103±46)和(1.20±0.32)mm2/M2,有明显增加(P<0.001)],末梢血氧饱和度[(71±11)%vs.(85±5)%]和血红蛋白浓度[(165±48)vs.(147±20)g/L]均显著改善(P<0.05)。所有33例患儿均完成了最终的根治手术。体外循环时间82~240分钟,平均(139±39)分钟,主动脉阻断时间42~180分钟(77±28)分钟,气管插管时间5~875小时,平均59小时,ICU滞留时间1~37天,平均5天。结论:根据肺动脉发育以及体肺侧枝情况设计个性化的治疗策略,能有效改善肺动脉发育,完成肺血的单元化供血,提高肺动脉发育不良型法洛四联症根治手术疗效。     

法洛四联症合并肺动脉闭锁外科治疗早期疗效分析

目的:探讨法洛四联症合并肺动脉闭锁(TOF-PA)外科治疗早期疗效。方法:回顾性分析1984年6月至2009年12月,我科对58例TOF合并PA外科治疗的临床资料,其中男性27例,女性31例;年龄6个月～29岁,平均(9.14±5.25)岁。按Castaneda分型,Ⅰ型28例、Ⅱ型11例、Ⅲ型9例及Ⅳ型10例。本组49例采用全麻中度低温体外循环下进行一期矫治手术,Ⅰ型采用跨瓣环的带单瓣的人工血管补片加宽,Ⅱ型采用右心室到肺动脉带瓣管道,Ⅲ型和Ⅳ型采用胸骨正中切口一期单源化和心内修复术。11例在全麻下行主动脉-肺动脉分流术。结果:术后早期并发症:心律失常9例、早期低氧血症7例、严重低心排出量综合征(低心排)5例、血红蛋白尿3例、灌注肺3例、气胸3例、多器官功能衰竭1例、毛细血管渗漏综合征1例、感染1例、肺不张1例及二次开胸止血1例。术后早期死亡10例,病死率5.8%,其余患者均治愈出院。术后1个月～15.5年复查,发绀消失,体质量明显增加。彩超复查未见残余分流,右心室流出道未见梗阻,肺动脉与右心室流出道之间无明显压力阶差。X线检查两肺血均有明显的增加,分布均匀对称。结论:TOF-PA一经诊断应尽快手术,加强术后监护及综合治疗措施,及时纠正术后低心排,积极防治术后心律失常等并发症,均为提高手术成功率的诸因素。外科手术应早期手术病死率低,中、远期心功能效果良好。     

非常规右心室切口根治法洛四联症

目的:采用非常规右心室切口手术的方法根治法洛四联症(TOF)。方法:2005年5月至2008年2月采用经右心房/肺动脉途径外科根治TOF患者83例。年龄5个月~25岁,平均(22.33±6.41)个月;体质量6.5~51.5 kg,平均(13.18±4.48)kg。28例采用单纯右心房切口;51例右心房加肺动脉联合切口,其中42例跨肺动脉瓣环补片扩大右心室流出道(右心室切口长5~20 mm);另4例经右心房及右心室小切口进行右心室流出道疏通。室间隔缺损经右心房切口连续缝合补片修复81例,另2例经右心房/肺动脉切口2侧修复。结果:术中停止体外循环后,65例采用直接心脏表面穿刺或通过漂浮导管检测,收缩期右心室压/体循环压(RVP/SAP)比值为0.3~0.81,平均(0.41±0.15);收缩期右心流出道残存压差(GRVP-PAP)为8~55 mm Hg(1 mm Hg=0.133 kPa)。术后早期死亡1例。结论:采用右心室微创的手术方法根治法洛四联症在技术上是可行的,操作简单,早期临床效果满意,对中晚期结果可能有潜在的益处。     

法洛四联症修补术后伴肺动脉返流患者的心肌组织多普勒成像Tei指数的研究

Tei指数(即心肌活动指数)是一种评价心脏收缩或舒张功能的新指标，既往多用于左心室功能评估。近来有研究表明，Tei指数还可运用于胎儿、儿童和成人的各种心脏疾病的右心室功能的评价。该研究对比研究了脉冲多普勒或组织多普勒方式在对法洛四联症修补术后伴肺动脉瓣返流(PR)患者心功能测定中的意义。     

肺血管发育不良性法洛四联症的分期治疗

目的:探讨肺血管发育不良性法洛四联症的治疗策略。方法:2007年11月至2013年12月,29例TOF因肺血管发育不良行分期手术,男性12例,女性17例。一期手术中位年龄15个月;体质量(10.7±5.0)kg;二期手术中位年龄36个月,平均体质量(14.7±5.1)kg。结果:全组无手术死亡。根治病例一期手术和二期手术平均间隔(21.8±9.4)个月。一期手术共计29例,结扎侧枝2例共5支,侧枝融合1例,McGoon比值由(0.78±0.13)增至(1.50±0.20)。两次手术间行肺动脉瓣球囊扩张5例。根治术后发生胸腔积液6例,灌注肺3例,肺不张2例,低心排出量综合征(低心排)与二次开胸止血各1例,均顺利康复出院。结论:肺血管发育不良性TOF分期手术安全有效,一期术后应根据肺血管发育情况适时行二期手术。     

法乐四联症肺血管发育评估方法的探讨

目的 :探索能更好地评估肺血管发育情况的新方法 ,为确定法乐四联症 (TOF)手术指征提供依据。方法 :采用 118例TOF患者的数字减影心血管造影胶片 ,用计算机辅助测量系统自动测量左、右肺动脉近、中、远端直径 ,分别计算Nakata指数 (PAI)、McGoon指数、改良PAI,再与术后情况进行相关分析。结果 :测量结果表明右肺动脉内径的最小值位于近端者有 6 2例 ,位于中部者 2 2例 ,位于远端者34例 ;而左肺动脉内径的最小值位于近端者有 4 6例 ,位于中部者 13例 ,位于远端者 5 9例 ;改良PAI与术后监护时间 ,呼吸机维持时间 ,正性肌力药物用量的相关性比PAI更高 ,PAI与术后情况的相关性比Mc Goon指数更高。改良PAI≤ 12 0mm2 m2 时 ,血液动力学很不稳定 ,低心排发生率与死亡率明显增加 ,术后综合情况差。结论 :改良PAI对TOF根治术的预后评估及TOF手术方案的选择有重要意义     

Coronary artery-pulmonary artery fistula in tetralogy of Fallot with pulmonary atresia

The treatment of a rarely described variant of tetralogy ofFallot with pulmonary atresia is reported. The case is uniquebecause the patient remained relatively pink although the pulmonaryblood flow came solely from a fistula between the left coronaryartery and the main pulmonary artery. The diagnostic and surgicalimplications are discussed and the literature briefly reviewed.The diagnosis should be borne in mind in patients who have pulmonaryatresia with ventricular septal defect and a continuous murmur.     

法洛四联症肺动脉瓣缺如的外科治疗

目的 介绍法洛四联症肺动脉瓣缺如的外科治疗经验。方法 1985年1月-2000年9月，共治疗法洛四联症肺动脉瓣缺如13例，男10例，女3例。全组均在中低温体外循环下行根治手术；补片关闭室间隔缺损；解除处理右心室流出道梗阻；扩张的肺动脉部分切除整形，6例肺动脉区植入瓣叶。结果 术后死亡1例，死亡率7.7%，术后并发症包括；低心排出量3例，心包积液1例，经治疗均痊愈。结论 儿童型选择性根治处理原则同普通法洛四联症，婴幼儿型需立即手术根治并植入肺动脉瓣或带瓣管道解除右室流出道梗阻，瘤样扩张的肺动脉做部分切除和折叠整形。     

Aberrant right coronary artery occlusion during the percutaneous pulmonary trunk stenting in a patient with tetralogy of Fallot

Aberrant coronary arteries are frequently observed in patients presenting with Fallot’s tetralogy (TOF). Before the complete surgical repair of the TOF, the percutaneously performed pulmonary trunk (PT) angioplasty is often performed in order to temporarily increase the pulmonary circulation, thus increasing the pulmonary vessel size, finally improving surgical outcome. This case reports a 12-year-old boy with a TOF insufficiently improved by surgical correction, in whom a PT angioplasty with stent implantation was complicated by an extrinsic compression of an aberrant right coronary artery (RCA) causing a myocardial ischemia with severe hypotension. The RCA, originating from the left anterior descending coronary artery, passed through the aortic root and the PT and was thus compressed by the PT-stent. Finally the RCA was successfully treated with standard coronary balloon angioplasty and stenting, improving myocardial perfusion and the hemodynamics of the patient, who finally died several days thereafter due to septic shock and massive pulmonary embolism.     

Long-term follow-up of homograft function after pulmonary valve replacement in patients with tetralogy of Fallot.

AIMS: To analyse the long-term outcomes after pulmonary valve replacement (PVR) in patients with a previous correction for tetralogy of Fallot. METHODS AND RESULTS: In a retrospective study, 158 adult patients with a diagnosis of tetralogy of Fallot, who had undergone a PVR after initial total correction in childhood, were identified from the CONCOR (CONgenital CORvitia) registry. All patients underwent 175 PVRs between June 1986 and June 2005. To analyse the predictors for homograft dysfunction and adverse events (death, reoperations, balloon angioplasty), Cox-regression analysis was performed. Overall freedom from significant homograft dysfunction was 66% after 5 years and 47% after 10 years. We could not identify predictors for combined homograft dysfunction. Event-free survival was 78% at 10 years and 68% at 15 years after PVR. Both early significant pulmonary regurgitation (PR) (HR 6.8, P = 0.017) and pulmonary stenosis (PS) (HR 4.0, P = 0.037) after surgery were associated with adverse events. When analysing direct post-operative PR or PS, we observed that in patients with severe, pre-operative PR, right ventricular aneurysm/patch resection resulted in a lower post-operative PR (mean difference grade 0.38 +/- 0.14, P = 0.01). Less significant post-operative PS was associated with a higher diameter of the homograft (HR 0.37, P = 0.006). CONCLUSION: While 47% of the patients in our study were free from homograft dysfunction at 10 years after PVR, event-free survival after PVR remained fairly good (78%). Significant residual lesions directly after surgery influenced event-free survival. A smaller diameter of the pulmonary homograft and severe pre-surgical PR were related to early homograft dysfunction after surgery.     

成人法乐氏四联症的外科治疗

目的 总结成人法乐氏四联症外科手术治疗的经验。 方法 从1996年10月至2003年12月对82例18周岁以上成人法乐氏四联症实施根治手术,最大年龄48岁。所有病例均经右室流出道切口完成疏通及室缺修补,室缺全部使用涤纶片修补,27例行跨环补片术,53例单纯补片加宽流出道,1例补片分别加宽主肺动脉和流出道,另有1例直接缝合流出道。 结果住院死亡1例,死亡率为1.2％。16例发生了并发症,包括因肺部感染二次气管插管1例,二次开胸止血6例,残余分流7例(均≤5mm),右室流出道残留梗阻2例(压差>60mmHg)。结论成人法乐氏四联症的根治手术可取得满意的临床结果。     

Echocardiographic and morphological correlations in tetralogy of Fallot.

AIMS: Our aim was to clarify the location and structure of the outlet septum relative to the free-standing subpulmonary infundibulum in the setting of tetralogy of Fallot and to examine its relationship to the other components of the subpulmonary outflow tract, determining their potential influence on clinical outcome. METHODS AND RESULTS: We studied prospectively 41 patients with tetralogy of Fallot (mean age 14 +/- 10.9 months) prior to surgical repair, and compared them with 15 patients undergoing closure of a ventricular septal defect associated with malalignment of the outlet septum but no subpulmonary infundibular stenosis (Eisenmenger ventricular septal defect), and 20 healthy controls. We also examined available autopsied hearts from cases with uncorrected tetralogy of Fallot (8) and Eisenmenger ventricular septal defect (13). Data were indexed for body surface area, and diameter of the tricuspid valve, respectively. The overall length of the subpulmonary infundibulum, including the extent of the muscular outlet septum, was significantly greater for patients with tetralogy of Fallot compared to normals (2.34 +/- 0.6 vs 1.46 +/- 0.34 cm/BSA0.5, P<0.001), whereas the difference between those with tetralogy of Fallot and an Eisenmenger ventricular septal defect was confined to the degree of narrowing of the subpulmonary outlet (0.43 +/- 0.22 vs 2.17 +/- 0.64 cm/BSA0.5, P<0.001). Within the tetralogy of Fallot group, there were linear relationships between deviation of the outlet septum (r= -0.61, P<0.005) and the diameter of the pulmonary valvar orifice (r=0.75, P<0.001), suggesting that growth of the pulmonary arteries may be related to this feature. When patients requiring a transannular patch as part of their surgical repair were compared with those not needing this procedure, differences were found in the diameter of the pulmonary valvar orifice and the pulmonary trunk, but not in the dimensions of the outlet septum. CONCLUSION: The position of the outlet septum in relationship to the remainder of the muscular subpulmonary infundibulum represents a hallmark of tetralogy of Fallot, permitting its differentiation from Eisenmenger ventricular septal defects and normal hearts.