带单瓣牛心包补片在法洛四联征疏通右室流出道中的应用研究

2007年6月—2010年6月我们在35例婴幼儿法洛四联征术中应用带单瓣牛心包补片跨瓣环疏通右室流出道,效果良好,现总结如下。1资料与方法1.1一般资料本组35例患儿中,男21例,女14例;     

活性带瓣主动脉补片重建成人重症法乐氏四联症右室流出道临床研究

重症法乐氏四联症(TOF)根治手术的关键是流出道的疏通、重建。目前，右室流出道(RVOT)重建多采用自体心包片跨肺动脉瓣环补片扩大右室流出道，因为肺动脉瓣环剪开及加宽后破坏了肺动脉瓣关闭的严密性，造成其关闭不全，致肺动脉瓣返流，加重了右室负荷，所以术后易发生心功能不全，难管理，病死率高。我们选择2000年1月至2003年6月收治的20例成人重症TOF患者应用活性同种带瓣主动脉片重建右室流出道，并与常规方法的20例进行对比研究，显示了满意效果。现报道如下。     

同种瓣与心包补片重建法乐氏四联症右室流出道后右心功能对比观察

法乐氏四联症(法四)根治术中右室流出道的处理是心血管外科中尚无一致性意见的问题[1].因右室流出道的处理直接影响右心功能,右心功能的异常常影响先天性心脏病术前及术后患者的存活率,而且右室功能受损必然会不同程度地影响左室乃至整个循环系统的功能.本研究分别应用同种带瓣主动脉补片及心包补片重建法四右室流出道,术中测压、术后多普勒超声心动图(UCG)随访,并进行对比,评价两种方法对右心功能的影响.     

法洛氏四联症根治术后肺动脉瓣置换术研究进展

法洛氏四联症根治术后肺动脉瓣置换术手术时间,目前尚存在争议。指征包括显著的肺动脉瓣反流合并重度右心室扩大（右心室舒张末容积指数〉150ml／m^2或〉2倍左心室舒张末容积指数）、持续性心房扑动／心房颤动或持续性室性心动过速及QRS间期〉180ms等。术后右心室功能迅速改善,表现为右心室容量、质量的减少,QRS间期缩短等。反流的肺动脉瓣常用同种移植物、生物瓣、机械瓣代替。经导管人工肺动脉瓣置换手术及镶嵌治疗可作为外科手术的补充治疗。     

8kg以下婴儿法洛氏四联症根治围术期治疗及并发症分析

目的 探讨8kg以下婴儿法洛氏四联症(TOF)一期根治术围手术期处理及并发症治疗。方法 2011年1月～2015年5月对81例8公斤以下婴儿术前完善检查及准备,术中细致操作,术后加强监护、个性化治疗等处理。结果 术后发生低心排6例(7.5％),死亡1例系严重低心排征并多脏器功能不全(1.2%);急性肾功能不全,腹膜透析2例(2.4%),室上性快速性心律失常5例(6.2%),肺不张4例(4.9%);膈神经麻痹1例(1.2%),心包积液2例(2.4%),均经积极治疗后恢复.结论有效的围手期治疗及积极处理并发症,是保证婴儿TOF根治手术成功的重要因素。     

小儿法洛氏四联症的围手术期护理配合

<正>法洛氏四联症是常见的一种复杂的先天性紫绀性心脏病畸形,主要病变是肺动脉和(或)肺动脉瓣狭窄、室间隔缺损、主动脉骑跨及右心室肥厚,其发病率占各类先天性心脏病的10%~15%,其中最常见的是主动脉口狭窄和室间隔缺损[1]。法洛氏四联症患儿出生后会出现不同程度的颜面、口唇、甲床青紫,生长发育迟缓,易感乏力,劳累后紫绀加重,呼吸困难与乏力常迫使患儿采取下蹲位休息,部分患儿由于严重缺氧会发生昏厥,甚至有癫痫抽搐,如不及时治疗     

法洛氏四联症修补术后室间隔残余漏介入封堵治疗

目的:报道法洛氏四联症修补术后室间隔残余漏介入封堵治疗体会。方法:总结2010-10-2018-11在我科住院的法洛四联症修补术后室间隔残余漏患者9例资料,介绍经导管行左心室造影及介入封堵治疗经验。结果:7例患者封堵成功,成功率77.78%,2例外科再修补治疗;无任何并发症发生,介入患者均5～6 d出院。结论:法洛四联症修补术后室间隔残余漏介入封堵治疗安全有效,并发症少,恢复快。     

法洛氏四联症矫治术后中远期肺动脉高压

【】目的 报道一组法洛氏四联症矫治术后中远期肺动脉压力及阻力升高的病例。方法2009.01-2011.04间我院行法洛氏四联症矫治术共181例(我院181例接受法洛氏四联症矫治术患儿),随访2-5年怀疑患出现肺动脉高压者并对其行右心导管检查最终确诊19例,分析单纯TOF矫治术后产生肺动脉高压的因素。结果19例患者在随访的2-5年内,绝大多数没有未出现明显临床症状。WHO心功能评定I/II级17例,III/IV级2例。问卷调查Borg呼吸困难评分为1-7分,中位数为4分。BNP检查为(11-＞300000)pg/ml,BNP的中位数为266pg/ml。右心导管检查示不同程度的肺动脉压力和阻力上升升高,其中轻度肺动脉高压16例,占绝大多数,所有患者肺小动脉楔入压均正常(6-10mmHg),肺动脉-右心室连续测压压力阶差小于10mmHg,右房压力属正常范围(6-11mmHg)。部分患者同时行肺小动脉造影,没有发现肺动脉高压特征性改变。无急性肺血管扩张试验阳性者。讨论部分法洛氏四联症矫治术后患者中远期会出现肺动脉高压及全肺阻力升高,其机制目前并不十分确定清楚,可能是一个多因素交叉的复杂过程。     

应用单瓣补片解除右室流出道梗阻

作者对单瓣补片解除右室流出道梗阻的应用价值,组织材料,适应证和外科技术,补片加宽标准等方面进行文献复习,指出应用单瓣补片加宽右室流出道可有效地防止肺动脉瓣返流,但仍存在着组织钙化、衰坏、右室流出道加宽标准等问题尚待深入研究。     

A new technique of transannular monocusp patch-repair of the right ventricular outflow tract in repair of Tetralogy of Fallot

Reconstruction of the right ventricular outflow tract (RVOT) is a key procedure in repair of Tetralogy of Fallot (TOF). The procedure creates pulmonary insufficiency (PI) that may compromise the right ventricular (RV) function, particularly during late follow-up. A simple way to reduce PI is to create a monocusp patch from xenografts, homografts, fascia lata, and autologous pulmonary artery or pericardium. Each of those has limitations. The autologous pericardial valve sewn on another pericardial patch is one of the earliest monocusp patches used clinically but loses anti-PI effect soon after the operation presumably due to degeneration or absorption of the monocusp. I have therefore designed and used a new technique (folded monocusp patch) to create a monocusp for TOF repair in children and adults. The technique has been used in nine patients of the 18 TOF patients who needed transannular patch-repair in total 74 TOF repairs. The monocusp patch-repaired patients had minimal or mild PI and good RV function beyond 8-12 months. The long-term results await follow-up and further study in multi-institutions.     

对法洛四联症中右心室流出道梗阻的再认识

目的:分析右心室流出道病理肌束的形成和构造,以期完善其外科纠治。方法:我院2002年1月至2007年3月选取50例法洛四联症(tetralogy of Fallot TOF)患者,年龄3个月~15岁,体质量6~38 kg。术中经右心室探查并描述右心室流出道的病理解剖。结果:圆锥隔前上移位是所有病例的共同特征,移位程度和主动脉骑跨均呈正比。所有患者都有隔、壁延伸。漏斗口位于圆锥隔下缘38例。低位漏斗口9例。存在弥散性肌束梗阻3例。所有患者的壁延伸连接于心室漏斗皱褶和游离壁。44例患者的隔延伸连接于室间隔。6例隔延伸和隔缘束间无间隙。3例弥散性肌束梗阻患者的调节束和隔缘束体部上移,挤压圆锥隔。离断解除延伸肌束,保留卵圆孔开放。所有患者有隔壁小梁肥厚,完全离断和切除。结论:法洛四联症中右心室流出道梗阻发生的病理基础是流出隔前、上移位和肌束延伸及隔壁小梁肥厚。术中准确识别病变肌束的性质和结构是维护术后良好心功能的关键。     

Right atrial area and right ventricular outflow tract akinetic length predict sustained tachyarrhythmia in repaired tetralogy of Fallot

Aims

Repaired tetralogy of Fallot (rtoF) patients are at risk of atrial or ventricular tachyarrhythmia and sudden cardiac death. Risk stratification for arrhythmia remains difficult.We investigated whether cardiac anatomy and function predict arrhythmia.

Methods

One-hundred-and-fifty-four adults with rtoF, median age 30.8 (21.9–40.2) years, were studied with a standardised protocol including cardiovascular magnetic resonance (CMR) and prospectively followed up over median 5.6 (4.6–7.0) years for the pre-specified endpoints of new-onset atrial or ventricular tachyarrhythmia (sustained ventricular tachycardia/ventricular fibrillation).

Results

Atrial tachyarrhythmia (n = 11) was predicted by maximal right atrial area indexed to body surface area (RAAi) on four-chamber cine-CMR (Hazard ratio 1.17, 95% Confidence Interval 1.07–1.28 per cm²/m²; p = 0.0005, survival receiver operating curve; ROC analysis, area under curve; AUC 0.74 [0.66–0.81]; cut-off value 16 cm²/m²). Atrial arrhythmia-free survival was reduced in patients with RAAi ≥ 16 cm²/m² (logrank p = 0.0001). Right ventricular (RV) restrictive physiology on echocardiography (n = 38) related to higher RAAi (p = 0.02) and had similar RV dilatation compared with remaining patients.Ventricular arrhythmia (n = 9) was predicted by CMR RV outflow tract (RVOT) akinetic area length (Hazard ratio 1.05, 95% Confidence Interval 1.01–1.09 per mm; p = 0.003, survival ROC analysis, AUC 0.77 [0.83–0.61]; cut-off value 30 mm) and decreased RV ejection fraction (Hazard ratio 0.93, 95% Confidence Interval 0.87–0.99 per %; p = 0.03). Ventricular arrhythmia-free survival was reduced in patients with RVOT akinetic region length > 30 mm (logrank p = 0.02).

Conclusion

RAAi predicts atrial arrhythmia and RVOT akinetic region length predicts ventricular arrhythmia in late follow-up of rtoF. These are simple, feasible measurements for inclusion in serial surveillance and risk stratification of rtoF patients.     

法洛四联症右室流出道疏通的临床经验

目的 探讨法洛四联症（Tetralogy of Fallot,TOF）根治术中右心室流出道疏通的最佳直径。方法 回顾性分析2011年1月至2016年12月我院心脏外科行TOF根治术的儿童病例308例,2011年1月至2013年12月201例患儿（A组）均采取右心室流出道疏通直径等同于标准肺动脉瓣环直径;2014年1月至2016年12月107例患儿（B组）采取右心室流出道疏通直径大于标准肺动脉瓣环直径3 mm。所有出院患者术后随访12~36个月。结果 A组有17例患者术后出现右心室流出道残余梗阻致低心排血量综合征（Low Cardiac Output Syndrome,LCOS）,多普勒测右心室流出道流速3.1~4.8（3.8±0.4）m/s,跨肺动脉瓣压差均大于50 mm Hg,其中6例右心室流出道流速超过4.5 m/s,再次手术行右心室流出道疏通术;术后早期死亡5例,随访余12例未发现右心室流出道残余梗阻进一步发展,且均较术后早期明显改善。B组术后无右心室流出道残余梗阻,跨肺动脉瓣压差均小于50 mm Hg,术后早期死亡2例。随访两组出院患者无三尖瓣、肺动脉瓣大量反流,生长发育良好,心功能均达Ⅰ~Ⅱ级。结论 TOF右心室流出道疏通直径等同于标准肺动脉瓣环直径造成术后残余梗阻发生率较高,大于标准肺动脉瓣环直径3 mm可有更好的疗效。     

Reconstruction of the right ventricular outflow tract with bovine pericardial monocusp patch

From November 1980 to May 1982, 19 patients underwent surgery in this institution for right ventricular outflow tract obstruction. Surgery was performed by placing a bovine pericardial monocusp patch across the pulmonary annulus. There was one hospital death (5.3%), and one late death. The follow-up ranged from 24 to 42 months (mean 32.5 +/- 5.3 months; total, 552 patient months). The condition of the remaining 17 patients is good. Repeat cardiac catheterization was performed in 11 patients and revealed that the right ventricular/pulmonary artery systolic gradient was reduced to 13.7 +/- 9.7 mm Hg postoperatively. Angiography showed a freely mobile, thin valve cusp. No calcification, aneurysmal dilatation, or shrinkage of the monocusp patch was observed. Our experience suggests that right ventricular outflow tract reconstruction with bovine pericardial monocusp patches can be safely performed with good hemodynamic results.     

Reconstruction of the right ventricular outflow tract in tetralogy of Fallot and pulmonary stenosis with a monocusp patch

In order to reduce postoperative pulmonary insufficiency (PI) a transannular monocusp patch was implanted in 14 patients with severe tetralogy of Fallot and hypoplastic pulmonary valve ring (group A). The results of left and right heart catheterization, obtained within one year of the correction, were compared to those of 9 patients, who received a simple transannular pericardial patch (group B). The mean maximal systolic pressure ratio between the right and left ventricle (A = 0.43 +/- 0.03; B = 0.49 +/- 0.04), the mean maximal systolic pressure gradient between the right ventricle and the pulmonary artery (A = 10.38 +/- 0.52 mmHg; B = 12.2 +/- 2.5 mmHg), and the degree of PI (A = 24.7 +/- 3.4%; B = 22.0 +/- 3.0% of total stroke volume) were not significantly different (p greater than 0.05) for the 2 groups. Although optimally implanted, the available monocusp patches cannot prevent or reduce postoperative pulmonary insufficiency in patients with severe tetralogy of Fallot. This experience suggests the need for a simple transannular pericardial patch if the pulmonary valve ring requires enlargement.     

法洛四联症右室心肌间质胶原改建与功能的关系

目的 :了解法洛四联症患者肥大右室心肌间质胶原改建与右室功能的关系。方法 :用生化方法定量分析 2 2例法洛四联症患者 (病例组 )和 8例非心血管及胶原系统疾病尸检者 (对照组 )的右室心肌间质中的胶原含量和 / 型胶原比值 ,并用右室穿刺法测定反映法洛四联症患者右室收缩及舒张功能的 dp/ dtmax及 - dp/ dtmax。结果 :病例组心肌每 mg总蛋白内含羟脯氨酸 (14.85± 2 .48)μg,较对照组 (7.5 5± 1.89)μg明显增多 (P <0 .0 5 ) ; / 型胶原比值为 5 .10± 1.84,较对照组 2 .2 7± 0 .5 8显著增高 (P <0 .0 5 ) ;病例组右室心肌间质胶原含量与术前、术后 dp/ dtmax无明显相关 ,而与术前、术后 - dp/ dtmax呈明显的负相关 ;其 / 型胶原比值与术前、术后dp/ dtmax及 - dp/ dtmax无相关。结论 :法洛四联症肥大右室心肌间质存在以胶原含量增多和 / 型比值增高为特点的胶原改建以及右室的舒张功能的下降 ,且后者主要与胶原含量增多有关     

Early outcome of tetralogy of Fallot repair in the current era of management

Background

Traditional use of trans-annular patch (TAP) to release right ventricular outflow tract (RVOT) obstruction during tetralogy of Fallot (TOF) repair may lead to a harmful pulmonary regurgitation. Different approaches have been used to release RVOT obstruction and spare the pulmonary valve (PV) function. In this study, we aim to evaluate the post-operative course of patients who had TOF repair in the current era that emphasizes on protective strategy of releasing RVOT obstruction and preserving PV function.

Methods

A retrospective study of all TOF cases repaired in our institute between March 2002 and December 2007 was conducted. Cases were classified into two groups; group I included patients that had a TAP, while group II included cases that had simple TOF repair without TAP. Group I was subdivided into two groups, group (A) which include patients who had TAP without a valve. Group (B) includes those who had TAP with a monocuspid valve (Contegra). We compared post-operative care and outcome of all groups.

Results

Eighty-three patients fulfilled the study criteria. There were 64 cases (77%) in group I, and 19 cases (23%) in group II. All children tolerated surgical repair and did well. We observed no statistically significant difference in the post-operative ICU care, complications rates and morbidity between all groups. There was no surgical mortality in all groups.

Conclusion

Children undergoing TOF repair had excellent short-term outcome with the current protective strategy aiming to spare valvular function, and conserving myocardial function. Applying a monocuspid patch technique did not show clear short-term benefits. Long term follow up is needed to evaluate future difference in different techniques.